SBJ -- Part One

SLE -- other antibodies

Anti-chromatin -- (anti-histone), drug induced syndromes anti-Sm -- specific to SLE, correlates w/ pleural disease, vasculitis Anti-U1RNP -- mild disease, raynaud's, restrictive lung disease Anti-SSA --subacute cutaneous lupus, neonatal lupus, sicca complaints, C2 and C4 deficiencies Anti-SSB -- neonatal lupus Anti-ribosomal phosphoproteins -- CNS vasculitis w/ psychosis

ANA

Antibodies detected by indirect immunofluorescence on fixed layers of HEp-2 cells (human carcinoma line) Screening for ANA w/ ELISA not trusted by most rheumatologists "blind" screening of patients can be misleading for diagnostic purposes Systemic autoimmune disease -SLE -scleroderma -MCTD -RA -Sjogren's -polymyositis/dermatomyositis -drug induced or discoid lupus -JIA Specific organ autoimmune disease -hashimoto's thyroiditis/grave's disease -autoimmune hepatitis -PBC -primary autoimmune cholangitis Other -chronic viral infection -malignancy (lymphoproliferative disease) -Drugs -False positive (women and elderly) ANA varies person to person and within person, not diagnostic

ANCAs

Antineutrophil cytoplasmic Abs Located in primary cytoplasmic granules of PMNs and monocyte lysosomes Binding of ANCA to PMN results in activation and oxidative burst and release of granule content and cytokines Used as serologic markers of vasculitides but also found in other inflammatory and infectious diseases Primary granules of interest: -myeloperoxidase (pANCA) -proteinase 3 (cANCA) Can characterize ANCA specificty by ELISA to look for target Ags (proteinase 3, myeloperoxidase)

Waste disposal hypothesis

Apoptotic cell eaten by macrophage normally In waste disposal hypothesis, immature dendritic cells interact w/ apoptotic cells, becoming mature dentritic cells that respond to the apoptotic cells Stimulate formation of autoreactive T and B cells, and the formation of autoantibodies by plasma cells

Sjogren's: extra symptoms

Arthralgias or myalgias, rarely erosive Pulmonary: cough, xerotrachea, bronchial hyperresponsiveness, pleural or parenchymal disease Renal: distal RTA, rare presenting symptom = hypokalemic paralysis, treat w/ KCl and alkalinzation (citrate) GI: dysphagia, dysmotility, webs, atrophic gastritis, discomfort, fullness, early satiety, MALT lymphoma Liver: autoimmune hepatitis, anti-mitochondrial Ab, PBC Large number of HepC patients will have evidence of sjogren's, those who do have higher prevalence of cryoglobulinemia, hypocomplementemia, and neuro involvement Vascular: purpura, immune complex mediated, raynaud's Thyroid disease: autoimmune thyroiditis Neurologic: most common extraglandular manifestation, peripheral neuropathy, treated w/ low dose TCA, gabapentin, or IVIG, progressive neuropathy might occur if necrotizing vasculitis, cranial neuropathy, transverse myelitis, treat w/ steroids, cyclophosphamide Lymphoma -- B cell, MALT, salivary glands, GI, lung, skin, thymus, thyroid

Cartilage

Cells in lacunae Abundant matrix Connective tissue covering Avascular***

Rheumatoid arthritis

Chronic, systemic autoimmune, inflammatory disorder Synovial tissue becomes inflamed, proliferates, forming pannus, which invades bone, cartilage, ligaments (Pannus -- inflammatory tissue that invades bone/cartilage -- destructive) Symmetric synovitis, bony erosions at joint margins Extraarticular manifestations More common in females than males, most in 30-40s, some in 60s

Anti-CCP

Cyclic citrullinated peptide Marker to identify RA earlier and w/ increased sensitivity Citrillination occurs naturally in body Deimination of arginine residues w/ in proteins to citrulline by PADI enzyme Citrullinated proteins found in synovial lining of RA patients -- target of autoimmune response Dying granulocytes release PADI which is then free to citrullinate proteins which are then presented as foreign Ag --> immune response

Chondroblasts

Derived from mesenchyme Found in inner lining of perichondrium Appositional growth Resemble fibroblasts (RER, euchromatic nucleus)

Lymphocytic sialadenitis

Diffuse collection of lymphocytes in parotid gland Eosinophilic deposit of thickened secretion in lumen of salivary duct Can erroneously be diagnosed as malignant

Arthritis w/ axial and peripheral involvement

Inflammatory -spondyloarthropathies -juvenile idiopatic arthritis -adult onset Still's -SAPHO syndrome Non inflammatory -osteoarthritis -acromegaly -ochronosis -spondyloepiphyseal dysplasia

Interstitial growth

No chondroblasts required Existing chondrocytes divide Isogenic cell nests are created (IMAGE)

OA treatment

No meds to stop or reverse disease Alleviate symptoms, increase function -NSAIDS, ASA, acetaminophen -Glucosamine chondroitin, limbrel -viscosupplementation -Joint replacement -PT -Topical capsaicin, diclofenac patches, lidocaine patches -Narcotics, steroid injections -Weight reduction (best therapy), exercise

Synovial fluid analysis

Observe color and turbidity, note amount obtained Send for cell count, crystals, gram stain and culture if possible If WBC over 2000, may be inflammatory or sepsis is extreme amounts Percent PMN: over 50%, inflammatory process, sepsis if over 75%, hemorrhage will be same as % in whole blood Noninflammatory will be clear, high viscosity, low volume Inflammatory will be low viscosity, higher volume, cloudy liquid Septic fluid mixed viscosity, opaque liquid of mixed color Hemorrhage -- high viscosity, high volume, red color Gram stain will usually be negative except in case of sepsis Don't contaminate fluid w/ steroids

RA: labs

RF autoAb (usually IgM); poor sensitivity for early RA Not specific for RA, many many other inflammatory, non inflammatory, rheumatic and non rheumatic diseases associated

Fibrocartilage

Rare Exists in areas of high stress -insertion of tendons and ligaments -pubic symphsis -intervertebral disc No perichondrium Type II collagen + type I collagen (for strength)

Elastic cartilage

Rare External ear, auditory canal, resp tract Always has a perichondrium Elastic fibers + type II collagen Elastic makes cartilage bendable Elastic plates, bundles of dark staining material on slide

Endochondrial bone formation

Resting zone -- hyaline cartilage at joint space Multiplication/proliferation zone -- clusters of chondrocytes together, isogenic cell nests Zone of hypertrophy -- large cells and lacunae Zone of calcification -- basophilic change, chondrocytes die and leave behind confluent lacunae spaces Zone of ossification -- Eosinophilc change, presense of collagen and true bone

Specificity of ANA

SLE -- w/ dsDNA, Sm, SS-A, SS-B MCTD -- w/ RNP Scleroderma -- w/ anti-centromere, topoisomerase I (Ab to scl-70), RNA pol III Polymyositis -- w/ Jo-1, PM-scl, P1-7, PL-12, Mi-2 -- order myositis panel Sjogrens -- w/ SS-A, SS-B

Ocular tests: sjogrens

Schirmer's test -- 5mm or less after 5 minutes Rose bengal -- red purple discoloration at lower portion of cornea indicative of abrasions from decreased tear production

Pathogenesis of SLE

Self antigen activates immune response Immune complex Ag-Ab deposition in tissues Abs typically present long before diagnosis or symptoms, Abs present in a predictable order ANA is usually first, +ANA raises suspicion for autoimmune disease Anti-SSA and Anti-SSB Abs can be present without +ANA

Pathogenesis: RA

Synovial tissue is primary target Early: edema, angiogenesis, hyperplasia of synovial lining and inflammatory infiltrates Late: hyperplasia (type A synovial cells), subintimal mononuclear cell infiltrate, extensive plasma cell, macrophage, lymphocytic infiltrate, pannus formation IMAGE -- Synovitis Pathology: synovitis, bone erosion, pannus, cartilage degradation and joint space narrowing

ACS2: Rubella

"German measles" Congenital rubella infection -acute viral upper resp infection in pregnancy -fever, headache, sore throat -maculopapular rash on face and trunk -suboccipital LAD Infant at risk for: deafness secondary to congenital rubella infection

Atopic dermatitis

"infantile eczema" Strong association w/ allergic rhinitis and asthma Occurs in 1/3 of children w/ personal or family hx IgE usually elevated, but not purely IgE mediated process Etiology unknown -- TH2? The "Itch that rashes," pruritis is a hallmark Infants: under 18 mo present w/ acute/subacute dermatitis on the scalp, face, post. neck, trunk and extensor aspects of extremities After age 2, presentation is more chronic, lichenified and scaling on face, neck, trunk, flexural aspects of extremities (antecubital and popliteal fossa) Prone to develop secondary infections (staph, viruses, fungi) Treatment: topical glucocorticosteroid or macrolide immunosuppressants, phototherapy for more resistant disease

Territorial matrix

(Capsule) Rich in GAGs, help maintain hydration status of matrix - important for diffusion of nutrients

Tinea versicolor

** Presents as hypo or hyperpigmented slightly scaly coalescing macules on trunk and upper arms** Eruption most common during summer months Oily areas of skin, sternal region, sides of chest, back Caused by **malassezia furfur**, fungus that grows in the most superficial layers of the skin Micro: "spaghetti and meatballs" or short hyphae and yeast appearance Treatment: -ketoconazole cream and shampoo -selenium sulfide lotion -topicals can be used as prophylaxis Oral meds: ketoconazole monthly

Scleroderma: autoantibodies

ANA usually present at time of raynaud's Nearly all systemic sclerosis patients are ANA+ Anti-centromere -- limited disease, increased risk for PHTN Anti-topoisomerase -- diffuse disease, increased risk of pulmonary fibrosis

Myopathy: autoantibodies

ANA+ (except IBM) Myositis specific antibodies (MSA) are insensitive but can suggest certain clinical features ANA- does not exclude MSA Myositis specific: Anti-Jo1 (anti-synthetase) is most common MSA Anti-Mi-2 Anti-signal recognition particle antibody Myositis associated: Anti-PM-scl Ab U1-RNP Ab Ku Ro/SSA

Bisphosphonates

Antiresorptive Acts on FPP which inhibits osteoclast activation Side effects: -GI problems -visual disturbance -acute phase reaction -severe bone, joint and muscle pain -osteonecrosis of the jaw -atypical femoral fractures (inhibition of bone remodeling -- repair of microfractures)

Joint exam

Appearance -- erythema, atrophy, deformities Swelling Tenderness Warmth Movement (active and passive)

Peripheral arterial disease

Arterial occlusive disease: tissue hypoxia and cell death -acute or subacute thromboembolism in small or terminal arterioles gangrene -chronic limb ischemia due to PAD -diabetic foot ulcers Ankle brachial index less than .5 severe .5 to .8 moderate .8 to 1 mild over 1 indicates calcified vessels in diabetic patient

JIA: psoriatic arthritis

Arthritis and psoriasis OR arthrtis and two of the following -dactylitis -nail abnormalities (pitting/onycholysis) -psoriasis in a first degree relative

Pregnancy outcomes in SLE

Assess existing disease activity, Ab status -poor outcome if renal or CNS activity immediately before pregnancy; thrombocytopenia worrisome First pregnancy has strong predicitive power Disease seems to flare postpartum Treatment of flares improves fetal outcome

Psoriatic arthritis: course and treatment

Associated w/ flares and remissions Younger age of onset, males and acute onset have more severe disease NSAIDs and topicals Methotrexate for skin and joint Sulfasalazine -- don't tolerate side effects well (GI upset) TNF blockers

RA: classification/lab studies

Must have 6 or more points on scale -serology (RF and anti-CCP Ab) -joint involvement -duration of symptoms -acute phase reactants (CRP, ESR) Lab studies -normochromic, normocytic anemia -Positive RF -Anti-CCP Ab -Elevated ESR and CRP -Some have +ANA -Normal urinalysis, renal, hepatic and metabolic functions -High WBC and PMN in synovial fluid, negative culture, high protein, low glucose

Classification of SNSA

Must satisfy one of 2 entry criteria Inflammatory spinal pain (4 of 5) -onset before 45yo -insidous onset -improved by exercise -morning stiffness -over 3mo duration OR Synovitis and 1 or more of following: -family hx -psoriasis -IBD -urethritis, cervicitis, acute diarrhea in past month -buttock pain -enthesopathy (inflammation where tendon inserts into bone) -Sacroiliitis

Intervertebral disc

Nuclus pulposis in middle -- stains clear, composed of GAGs and water Annulus fibrosis around -- firbrocartilage found here Pockets of hyaline cartilage between bone and disc

HSV - treatment

Number of outbreaks w/in a given amount of time will govern therapy -acyclovir, valacyclovir, famciclovir -daily suppressive therapy if needed Sunblock daily on lips Recognition of possible triggers Counseling to avoid spreading

Appositional growth

Occurs at edges Osteoblasts required Inner chondrogenic layer of perichondrium

Keratin formation

Occurs in stratum granulosum Keratohyaline granules and lysozomes break down cellular components into keratin

JIA: oligoarthritis

Asymmetrical arthritis in four or fewer joints Large joints, typically knee, ankle or small joints of hands Females > males Early childhood onset (1-3 yr) 50% have chronic iridocyclitis +ANA increases risk of silent uveitis (increased risk of blindness) Joint residua rare, some have ocular damage from iridocyclitis If after 6mo greater than 4 joints invovled --> extended oligoarthritis, if still less than 4 --> peristent oligoarthritis

Calcium pyrophosphate dihydrate (CPPD)

Asymptomatic Various clinical manifestations -pseudogout -osteoarthritis -polyarticular inflammatory arthritis (pseudo RA) -pseudo-neuropathic arthropathy Can be idiopathic, hereditary, or metabolic: -hyperPTH -hemochromatosis -hypothryroid -hypomagnesemia -hypophosphatasia Increased incidence w/ age and preexisting joint damage Radio: punctate and linear densities in articular hyaline or fibrocartilage Crystals: Rhomboid/rod shaped Weakly, positively birefringent (blue when parallel to compensator) Treatment: Large joints; aspiration NSAIDS Occaionally colchicine Corticosteroids Possibly plaquenil

Fusiform excision

Atypical lesion, carcinoma, melanoma Ellipse around entire lesion that can be visualized (when you don't want to leave the lesion)

Sjogren's syndrome

Autoimmune disease w/ stron autoantibody response Persistent EBV (salivary glands site for latent virus) RF positive in majority of patients but is not part of criteria SS requires presence of 4/6 criteria, one of which must be positive biopsy or autoantibodies Criteria 1 -- symptoms of dry eye 2 -- oral symptoms (dry mouth) 3 -- ocular signs (schirmer's test, rose bengal score or other ocular dye test) 4 -- Histopathology (focal lymphocytic sialadenitis) 5 -- Salivary gland involvement 6 -- Autoantibodies Exclusions -prior head or neck radiation -hep C -HIV or AIDS -preexisting lymphoma -sarcoidosis -GVHD -anticholinergic drugs

Alopecia: pathogenesis

Autoimmune form of hair loss T cells attack hair bulb Pigmented hairs affected more severely than non -first to shed -regrowth is often non pigmented **Associated w/ vitiligo**

Dermatomyositis: biopsy

B lymphocytes and CD4 lymphocytes close to vessels Perifascicular atrophy (capillary depletion and dropout)

JIA: systemic onset

Onset: persistent intermittent fevers w/ or wo rheumatoid rash or other organ involvement, arthritis must be present for diagnosis Large and small joints (knees, wrists, MCPs, PIPs, DIPs, ankles, MTPs, elbows, hips, C spine, TMJ, shoulders) Males ~ females, any age Extra: high fever, rash, hepatosplenomegaly, polyserositis, lymphadenopathy, abd pain 20% severe arthritis Tx: prednisone and disease modifying agents depending on symptoms

Essential mixed cryoglobulinemia

CGs are cold precipitable monoclonal or polyclonal IGs, sometimes associated w/ vasculitis Also: -multiple myeloma -lymphoproliferative disorders -CTD -liver dz -Hep C infection -- VAST MAJORITY OF MCG Sx - palpable purpura, arthralgias, weakness, neuropathy, glomerulonephritis Labs: circulating cryoprecipitates, RF, hypocomplement, ESR ^, Hep C

Lupus and complement

CH50 -- ability of serum to lyse 50% of standard sheep blood suspension C3, C4 -- base line level; C4 more sensitive to minor episodes of complement activation of classical pathway C4 allotypes C3a, C5a, C4d, C3d, MAC (C5-C9)

Lupus and complement

Opsonize particles for phagocytosis Activates leukocytes Assembly of MAC In SLE, immune complexes stimulate classical pathway, hypocomplementemia occurs (C3 and C4) Complement deficiency may predispose to SLE

Acne

Oral and topical antibiotics: tetracyclines, sulfa, PCN -topical washes and creams: benzoyl peroxide, retinoids -intralesional corticosteroids -isotretinoin (accutane) -photodynamic therapy -chemical peels

Primary skin lesions

Original or most characteristic lesion ID is most important part of physical exam Macules Papules Nodules Vesicles Pustules Patch Plaque Tumor Bulla Wheal

Osteoarthritis: radiology

Osteophytes -- bone spurs at joint margins Nonuniform joint space narrowing Subchondral cysts -- Subchondral sclerosis Malalignment IMAGE -- irregular joint space narrowing of DIPs, PIPs, osteophytes, sclerosisi, narrowing and subluxation of first MCP

SLE: outcomes

Overall risk for symptomatic atherosclerosis may be increased as much as 50x in women w/ SLE Adequate anticoagulation in pts w/ antiphospholipid while monitoring for bleeding Minimize adverse outcomes -monitor for infection (immunosuppressive therapy) -monitor for drug toxicity -BP control -electrolyte monitoring -osteoporosis -monitor for hyperlipidemia

MCTD

Overlap syndrome w/ features of SLE, SSc, and polymyositis Ab to U1-RNP Clinical: -arthritis/arthralgias -raynaud's -decreased esophageal motility -impaired pulmonary diffusing capacity - pulm fibrosis -swollen hands -myositis -lymphadenopathy -PHTN -sicca Prognosis worse than lupus, PHTN most common cause of death, can have renal involvement (SSc renal crisis or membranous nephritis) Treatment depends on organ involved, disease can evolve

Pressure ulcers: common errors

Overly aggressive debridement Use of -cidal agents on granulation tissue Overuse of antibiotics -creates multiply resistant organisms Systemic antibiotic w/o evidence of systemic infection

Medium sized vasculidities

PAN Kawasaki's disease

Patellofemoral pain syndrome

Pain in anterior aspect of the knee Adolescence Girls > boys Sx: pain w/ descending stairs, walking downhill -need to sit w/ legs straight -patellar tenderness on compression -weak quadriceps -small effusion

Joint examination: pain patterns

Pain in central part of ROM but not extremes: implies structure being pinched Pain w/ repitition: vascular issue?, degree of arterial patency Pain at extreme of range: compression or tension of structures around the joint, in the joint

Nodule

Palpable, solid, round lesion which is centered in the dermis or subcutaneous tissue, diameter over 1 cm

Subluxation

Partial dislocation of joint

Vasculopathic pattern

Path: histological changes in cutaneous blood vessels Ex: leukocytoclastic vasculitis Histo: fibrinoid necrosis, leukocytoclasis and erythrocyte extravasion Palpable purpura, not a disease, a reaction pattern, caused by something else Most common site, lower extremity

Spongiform/eczematous pattern

Path: intraepidermal intercellular edema Include a group of diseases w/ common morphology: erythematous and papulovesicular when acute; erythematous and scaling when chronic Ex: atopic dermatitis, contact dermatitis, nummular dermatitis, seborrheic dermatitis, and stasis dermatitis

DISH

Diffuse idiopathic skeletal hyperostosis Usually SI joints are spared Apophyseal joints are not anykylosed Osteophyte formation and flowing calcification Disc height maintained At least 4 continous vertebral bodies are ossified (classically right side thoracic) Extraspinal involvement w/ hyperostosis

Telangiectasia

Dilated superficial blood vessel

Scleroderma: cardiac

Disturbances of conduction system of heart Arrhythmias Heart failure Pericardial effusion/pericarditis

Sicca differential

Dry eyes/ dry mouth --> not indicative of sjogren's HIV can cause DILS (diffuse infiltrative lymphocytosis syndrome), looks like SS but w/o anti SSA/SSB Abs, may have RF or ANA -- immunostain of salivary glands will have high CD8 instead of CD4 DDx -other viruses: mumps, EBV, HTLV-1 -GVHD -sarcoid -amyloid -radioiodine therapy -aging -fibromyalgia like syndromes -lipodystrophy -bulemia -dyslipoproteinemia -hemochromatosis

Atypical melanocytic nevi

Dysplastic melanocytic nevi, clarks nevus Usually benign moles Generally larger than other moles May have color that is not uniform May have irregular or indistinct borders May resemble melanoma May indicate increased risk of melanoma Can run in families, affect more caucasians 3/5 criteria: -poorly defined borders -irregular border -irregular pigment -background erythema -larger than 5mm

Myopathy: workup -- muscle eval

EMG Biopsy -- gold standard MRI w/ T2 weighted fat suppressed images can identify edema or active muscle inflammation (help to localize site for biopsy)

Gout: radiographic features

Early -- normal Erosive overhaning edge "rat bite" Joint space is preserved No juxta-articular osteopenia

2 Wheeled walker

Easier to move forward, doesn't require as much balance/upper extremity strength Help with propulsion and balance (as opposed to a four wheeled walker) Ex: cognitively intact w/ parkinsons, weak in legs after extended ICU stay, cerebellar ataxia, bilateral arthritis of legs and arms

PAN labs

Elevated WBC Anemia Elevated acute phase reactants Hypergammaglobulinemia Hep B **ANCA -- MPO **

Ankylosing spondylitis -- clinical

Enthesistis -- ischial tuberosites, greater trochanters, spinous processes, costochondral/manubri ostostenal, iliac crests, achilles, plantar fasciitis Back pain -- deep in gluteal region, dull, difficult to localize, insidious onset (SI joint, alternating buttock pain, low back stiffness wakes from sleep and is worse in AM Joints -- hip and shoulder girdle most frequent, in boys hip disease is most common, knee w/ intermittent effusion, TMJ involvement in some Extra skeletal - fatigue, weight loss, low fever, acute anterior uveitis, CV (aortitis, conduction abns, aortic regurg, cardiomegaly, pericarditis) Neuro -- spinal fracture C5/6 and C6/7 most common, cauda equina syndrome Renal - IgA nephropathy, rare - amyloid Pulm - rare, progressive UPPER lobe fibrosis of lungs, restricted chest wall movement

JIA: enthesitis related arthritis

Enthesitis, arthritis and at least two of following: -sacroiliac joint tenderness -inflammatory spinal pain -HLA-B27 -arthritis in male over 6yo -family history of (anterior uveitis, SA, or sacroiliitis w/ IBD) -Anterior uveitis w/ pain redness or photophobia

Punch biopsy

Eruptions/ inflammatory conditions Punch, twist, cut, suture

Septal panniculitis

Erythema nodosum Septal panniculitis -- inflammed septum between lobules of fat, contain number of multinucleate giant cells in addition to lymphocytes and eosinophils Clinical: painful, erythematous, non ulcerated deep nodules, symmetric, localized to anterior tibial surface Evolve from bright red to brown-yellow May also have fever, fatigue, arthralgias More common in women in 2-4th decade but can occur at any age, lesions subside after 2-6 weeks Reaction pattern to infections (strep, TB, histo, coccidiomycosis), medications (OCPs, sulfas), systemic disease (sarcoid, IBD), neoplasm (lymphoma/leukemia), idiopathic in most cases Treatment: treat underlying disorder, NSAIDs, corticosteroids, potassium iodide

Crystalopathies

Gout -- monosodium urate monohydrate Calcium pyrophosphate dihydrate Basic calcium phosphates Calcium oxalate

IBM (inclusion body myositis)

Gradual onset, years Older age, more in males Distal and proximal weakness, atrophy of anterior thigh, prone to falling Distal polyneuropathy, no extraskeletal manifestations Less ANA+, no MSAs CK may be normal Poor response to therapy Micro: lined or rimmed vacuoles, can look like polymyositis, tubulofilaments in cytoplasm may be seen on EM

ANCA associated small vessel vasculitis

Granulomatosis w/ polyangiitis (Wegener's granulomatosis) Churg Strauss Syndrome Microscopic polyangiitis

Atypical nevus syndrome

Often have over 100 moles at least some of which are unusual (atypical) Moles vary in size, location, and color usually larger than normal moles (5mm or more) Have irregular borders and pigmentation Management: -changes in appearance are serious -itchy, tender, or painful moles should be evaluated -patients should have yearly skin exams -can perform self exams w/ a partner -sun protection a must from early age -should avoid tanning beds (as should everyone)

Culture of chronic wound

Only after wound infection has been diagnosed clinically 100% of wounds are contaminated/colonized but not necessarily infecting/harming host

Estrogen replacement

Only works while you take it If you're going to use it, use it early and assess benefits/risks yearly

Primary raynaud's

Onset is 15-30yo, more common in females, can be familial Symmetric, no evidence of vascular disease, no tissue gangrene, digital pitting, or tissue injury Negative nailfold capillaroscopy Negative ANA, normal ESR May remit over time 15-20% of patients w/ RP w/ autoantibodies will develop a defined CTD w/in 2 years

Clues suggesting secondary raynaud's

Onset over 40yo, male, digital ulcers, asymmetric attacks, abnormal labs, ischemic signs and symptoms proximal to fingers and toes, abnormal nailfold capillaroscopy

Baker's cyst

Posteriorly on knee, occur in RA, can rupture during violent flexure of knee, can mimic acute DVT

Actinic keratosis

Precancerous lesion made up of keratinocytes from sun exposure -some will develop into SCC if untreated -risk factors for progression: persistence, h/o of skin cancer and immunosuppression Appear as rough, slightly scaly, pink macules -sandpaper like -usually 3mm-1cm, most under 6mm, larger lesions should raise suspicion for BCC or SCCIS

Erythema multiforme

Precipitating factors: HSV, mycoplasma, histo, drugs, +/- radiation Abrupt onset, target lesions, dusky center Hands/forearms/trunk/feet/palms and soles, oral erosions Recurrences common Tx: treat cause, antihistamines, topicals, prevention: sunscreen and aciclovir for HSV; self limited

Congenital nevi

Present since birth Can be dome shaped, mammilated, have hairs Risk of development of melanoma is same as general pop until become large (over 20cm then risk is greatly increased) Measure along longest axis, then measure perpendicular axis

Pulmonary involvement of myopathy

Lung is most common extra muscular target Dyspnea from interstitial lung disease or diaphragmatic and intercostal muscle weakness PFTs will look restrictive HRCT of chest -- ground glass or honeycombing/fibrosis -nonspecific interstitial pneumonitis -Organizing pneumonia -usual interstitial pneumonitis -- worse May have secondary PHTN

ANCA vs ANA

Lymphocytes on smear may help If fixed in formalin, pANCA may give a stain, whereas ANA will not

Takayasu's arteritis

Main involvement of aortic arch and its branches **Adolescent girls and young women** Most common in Asia Sx - malaise, fatigue, fevers, night sweats, anorexia, weight loss, absent pulses (in subclavian distribution), HTN Lab: elevated ESR, mild anemia, elevated immunoglobulins **Diagnosis made by angiography: irregular vessel walls, stenosis, poststenotic dilatation, aneurysm formation, and occlusion**

Erosion

loss of some or all of the epidermis; heals w/o a scar

Valgus

lower limb deformity where distal part is directed away from midline

Varus

lower limb deformity where distal part is directed toward midline

Effects of ischemia on tissue

Prevents macrophage, fibroblast, growth factor migration to the wound Ulcer paradoxically clean and dry, no inflammation, no proliferation Gangrene is NOT an infection: necrosis Treatment Wet gangrene -- early surger for odors or painted w/ a dessicant Dry gangrene -- allowed to autoamputate Therapy: -protect at risk watershed tissue -restore perfusion -manage pain -never send wound swab culture

Disseminated fungal infections

Primary site of infection: lungs, dissemination in compromised patients Nort american blastomycosis -most common deep fungal to cause skin disease -paulopustules or verrucous plaques -present in 80% of disseminated dz Cryptococcosis - CNS and skin two most common sites of disseminated infection -AIDS patients: presents as molluscum like lesions on face Fusarium: opportunistic infection in neutropenic patients; vesiculonecrotic lesions

Syphilis

Primary: chancre -- painless ulceration Secondary: reddish papules and plaques on palms and soles Tertiary: systemic: CNS, heart, vasculature, skin and bone Neurologic findings: marcus gunn pupils, tabes dorsalis

Lyme disease

Primary: erythema chronicum migrans -red papule at site of bite that expands peripherally as a nonscaling, palpable band that clears centrally Secondary: bells palsy, pericarditis, mononeuritis, malaise/fever/chills Tertiary: acrodermatitis chronica atrophicans - usually years after -rheumatologic, cardiac, and neurologic problems

Treatment: sjogren's

Regular dental exams External moisture replacement -artificial tears -collagen or silicone plugs -permanent electrocautery -artificial saliva -humidifier Secretogogues to stimulate endogenous secretions Caution if asthma, narrow angle glaucoma, acute iritis, severe CAD, biliary dz, nephorlithiasis, diarrhea, ulcer dz -pilocarpine -cevemilline NSAIDS for arthritis/pain Low dose steroids for flares Higher dose steroids or immunosuppressives if more severe organ involvement

RA: lab screening

RF and anti-CCP can be present prior to development of clinically apparent RA Presence of both RF and anti-CCP in individual w/ 2 or more relatives w/ RA gives 100% predictive value of development of RA Predict persistent RA requiring long term therapy Together, they predict severe, erosive and deforming arthritis w/ likely poorer functional outcome

Psoriatic arthritis - labs

RF negative Elevated ESR Elevated uric acid Diagnosis is clinical -- look for hidden psoriasis

AS - physical exam

ROM of low back -forward flexion - Schober test (10 to 15 cm) -extension -rotation -lateral flexsion Reduced chest expansion -level of 4th ICS -Maximal expiration followed by max inspiration (less than 5cm is abn) Test for sacroiliac pain -- negative either very early or late C-spine -- stand against wall, extend neck to touch wall, normal can touch wall

Alopecia areata

Rapid loss of hair **One or more round or oval patches on the scalp or beard** **Nonscarring, non scaly smooth hairles patch** May effect eyebrows, eyelashes, or intranasal hair Spares white hairs **Nail pitting may occur** Relapsing recurring course in most -isolated episode w/ single patch in some -severe course rare: --totalis - full scalp loss --universalis -- full body loss of hair, including eyelashes and nasal hairs

Merkel cell CA

Rare, aggressive, primary skin cancer w/ NE differentiation More caucasians, usually over age 75, 2 yr survival is 50-70% women doing better than men Chromosome 1 abns Risks: chronic exposure to solar UV radiation, polyomavirus? Clinical: solitary, dome shaped nodule or firm plaque, red violaceous or purple, head and neck Histo: dermis, intraepidermal component may also be present, purely intraepidermal rare, -trabecular pattern w/ interconnecting strands of tumor cells in dermis, w/ formation of cellular aggregates resembling glands or neural rosettes Management: -wide local excision -selective lymphadenectomy -role of chemotherapy/radiation unclear

Eccymoses

Red to purple patches resulting from inradermal hemorrhage; non palpable, non blanching, > 1 cm in diameter

Joint examination: reflex and cutaneous distribution assessmnt

Reflexes, sensation If there are positive signs/symptoms, a "neuro exam" must be performed -Correlate w/ results of objective exam

Cardiac involvement: myopathy

Relatively uncommon If present increased risk for mortality Electrical disturbances/arrhythmias CK-MB level unreliable Use cardiac troponin I Myocarditis?

Lupus flare

Remission of all activity is unusual Flares can vary greatly even in the same patient Can be very difficult to assess

Scleroderma: renal

Renal crisis used to be most common cause of deat in SSc prior to ACE inhibitors Can still occur in early diffuse disease: malignant HTN, headaches, heart failure, proteinuria, hematuria, creatinine rise Treatment is ACE inhibitors, BP control

Osgood-Schlatter disease

Repeated trauma to tibial tuberosity leading to microavulsion fx Adolescent athletes Boys > girls Sx: -pain over tibial tubercle --> worse w/ exercise -unable to kneel secondary to pain -tenderness, swelling of infrapatellar tendon X ray: -soft tissue swelling -enlarged/fragmented tubercle Treat symptomatically

Joint examination: neuro tension test

Reproduces neural symptoms -- neural tissue is at fault head flexed, slumped over, extend legs, see if painful response is elicited

Osteoclast

Responsible for bone resorbtion Stains intensely pink (mitochondria), polynucleated Attaches firmly to bony surface and erodes it away, leaves a lecunae (sign of active resorbtion)

Diabetic dermopathy

Result from changes in small blood vessels that supply skin and minor leakage of blood products from the vessels into the skin light brown or reddish, oval or round, slightly indented scaly patches AKA pretibial pigmented patches

Epidermoid cyst

Results from plugging of follicular orifice or epidermal implantation Most common cutaneous cyst Dermal compressible nodule w/ central punctum Freely movable, but attached to normal skin above them Usually on face/upper trunk Cheesy white contents can be expressed that is composed of macerated keratin Pungent odor May rupture and induce vigorous inflammatory (non infectious) response Only way to remove is surgical, if fragments of wall are left the cyst may recur

Pacinian corpuscles

Round, look like an onion, central unmeylinated axon, and membranes surrounding derived from endoneurium Lymph like material between layers of membrane Important in deep pressure and vibration sensation, found in hypodermis

Squamous cell carcinoma

SCC in situ -- intraepidermal atypical squamous cells not showing maturation and appear disordered Invasive SCC - irregular sheets of atypical squamous cells originating from the epidermis and invading the dermis Keratin pearls Second most common More common in blacks than BCCs Risks: UV exposure, HPV, chronic wounds, burn scars, radiation Presents as pink/red indurated crusted papules or plaques or scaly non healing patch Usually slow growing, do have higher risk of metastasis (higher risk at lips, ears, genitals, chronic wounds and scars, recurrent lesions, or immunosuppressed patients)

Discoid lupus erythematosis

Scarring photodistributed rash -heals w/ atrophy, scarring, dyspigmentation, telangiectasias -~5% go on to develop SLE, esp patients w/ disseminated disease or high titer ANA

SERM's

Selective estrogen receptor modulator raloxifene, tamoxifen May also have protective effect against breast cancer

Anti-dsDNA

Specific to SLE Correleates w/ renal disease, CNS disease, vasculitis, hypocomplementemia, indicates active disease Assays: Crithidia luciliae -- high titer very specific for SLE and can correlate w/ disease activity Farr, ELISA (more sensitive, can give + for ssDNA), high titers specific for SLE

Dermatofibroma

Spindle cell proliferation in the dermis that looks like a wad of scar tissue Common fibrous tissue in dermis Red/brown firm papule or nodule, can have a yellow hue Predilection for extremities of young adults (legs > upper arms > lateral trunk) Elevated --> slightly depressed Firm papule is attached to overlying epidermis -- dimple sign No malignant potential Dimple sign, can have a peripheral rim of darkening

Pressure ulcer: diagnosis

Stage 0 - red, blanching "post ischemic hyperemia Stage I - non blanching erythema -blue/purple discoloration in dark skin Stage II - superficial erosion Stage III - subcutaneous affected Stage IV - deep tissues involved Unstageable: intact, hard eschar so depth of wound cannot be determined and is presumably 3 or 4

Acquired nevi

Start to appear in infancy Increase in number until age 30 Sun exposed > non exposed Decrease in number later in life Mature through stages: flat spot --> brown bump --> flesh colored bump --> compound --> intradermal Can grow w/ child, can become darker and/or elevated w/ trauma New lesion after 50y should be biopsied

Venous stasis ulcers

Stasis ulceration and dermatitis (4 "ins") Inflammatory cells and substances lead into tissue by venous, lymphatic incompetence Interstitial fluid accumulates necrotic debris, bacteria, chemotactic agents Interstitial pressure exceed arterial capillary pressures, ischemia Impedes fibroblast growth factor penetration into dermis Therapy: Reduce edema by compression Clean superficial slough w dressings Gentle debridement NO WET TO DRY Treat co-existent cellulitis

Leukocytoclastic vasculitis (small vessel)

VASCULITIS Viral hepatitis Autoimmune (SLE, SS, RA) Strep, staphy (henoch shonlein purpura) Cryoglobulins UC, urticarial vasculitis Lymphoproliferative disease (hairy cell leu) Idiopathic Thiazides, phenothiazines Iodides Sulfas

Livedo reticularis

Vascular condition characterized by purplish discoloration of the skin, usually on legs Lacy or net like, may be aggravated by cold exposure Can be associated w/ lupus, anti-phospholipid syndrome, initial presentation of calciphylaxis and medications (hydroxyurea)

Juvenile dermatomyositis

Vasculopathy of skin and muscles causing inflammation -Muscle weakness -skin rash Symmetric weakness of proximal muscles Characteristic cutaneous changes: -heliotrope rash (w/ periorbital edema) -Gottron's papules (IMAGE) -elevated muscle enzymes (CK, aldolase, LDH, neopterin) -EMG: myopathy and denervation -muscle biopsy: necrosis and inflammation XRAY - IMAGE

Freckle (ephelis)

Very common Multiple red brown macules on face and shoulders -often genetic in fair skinned individuals -darken easily w/ sun exposure Histology -increased melanin in basal layer, not melanocytes -normal or decreased number of active melanocytes -NO elongation of rete ridges or nesting of melanocytes Ddx: -lentigo simplex -solar lentigo -cafe au lait spots

Verruca vulgaris

Warts Destructive: liquid nitrogen, salycylic acid, urea, cantharidin, all w/ or w/o occlusion, laser Immunomodulation: imiquimod, candida or mumps Ag, DCP (dicyclophenone) therapy, cimetidine

Boutonniere deformity

Weakening of central slip of the extensor tendon and palmar displacement of the lateral bands IMAGE -- pinkie finger

Myopathy: presentation

Weakness > pain Myalgias may be present w/ exercise Proxymal dysphagia, nasal regurg, pulmonary aspiration (poor prognostic sign/ emergency) Hoarseness, dysphonia

Features pointing away from autoimmune myopathy

Weakness related to exercise, fasting Facial weakness Family history of muscle disease Neuropathy, fasciculation, cramping Early muscle atrophy or hypertrophy Lack of CTD symptoms, serologies Enzymes less than 2 or greater than 100x normal

cANCA

Wegener's granulomatosis Granular staining of cytoplasm of PMN and monocyte lysosomes methanol or ethanol fixed, cANCA pattern is same Ab directed against proteinase 3

Benign neoplasms

Well differentiated Few mitotic figures Usually expansive growth Slow growth May cease growing Usually encapsulated No metastasis Only slight harm to host due to location or complication

Pressure ulcer complications

amyloidosis Endocarditis Abscess and sinus tract formation Fistulas Pseudoaneurysm SCC Prolonged hospitilization, institutionalization, pain, loss of autonomy, depression, documentation, documentation

Palpable purpura

circumscribed, purple, palpable, non blanching hemorrhage into skin w/ inflammation of dermal blood vessels less than or equal to 1 cm in diameter Clinical term for the histologic finding of leukocytoclastic vasculitis

Dermatophytoses - epidemiology

common after puberty except for tinea capitis which is more common in children Risk factors: children include exposure to other peoples hats, brushes and barber instruments Males are affected more than females for tinea pedis and cruris

Mohs cutaneous micrographic surgery

treatment of various types of skin cancer skin preservation w/ nearing 100% margin evaluation of excised tumor Cosmesis and clearance Used in treating: -recurrent tumors -histologically infiltrative tumors ->2cm tumros, ill defined, facial (particularly embryonic fusion areas) -prior history of radiaiton, transplant patient -anatomically challenging areas: facial, acral

Scoliosis

Lateral curvature of spine

Scleroderma: pulmonary

Leading cause of death in SSc Diffuse at risk for fibrosis -ground glass appearance on HRCT -PFTs may show restrictive pattern Limited at risk for PHTN =secondary to pulm fibrosis or vasculopathy -PFTs w/ isolated decrease in DLCO -screen w/ echo, may need cardiac catheterization to diagnose -initially is silent, later symptom is dyspnea

Verruca vulgaris

Lesions present as elevated, rounded papules and plaques w/ a rough surface -tiny black dots representing thrombosed capillaries may be seen on surface May appear anywhere on skin, dermatoglyphics not present Extremely common between 5-20 yo 90% resolve spontaneously w/in 5 yrs Can spread by auto-inoculation

Derm: secondary lesions

Lesions that evolve or develop as natural progressions from primary lesions or from events like scratching, irritation, and secondary infection of primary lesions May be only type of lesion present Scale Erosion Ulcer Crust (scab) Fissure (crack, cleft) Excoriation and abrasion (scratch mark) Atrophy Scar

Behcet's syndrome

Leukocytoclastic venulitis w/ fibrinoid necrosis **All sized vessels may be involved ** Mediterranean, middle east, far east, males have more aggressive course Recurrent apthous ulcers: painful, shallow, or deep w/ central necrosis, genital ulcers less common Skin involvement: -folliculitis -erythema nodosum -acne like exanthem -vasculitis -abn pathergy test -- rxn after intradermal saline injection Eye: retinal vessel occlusion, optic neuritis, bilateral panuveitis w/ scarring --> blindness Arthritis DVT GI - ulceration SVC obstruction Aortitis or peripheral arterial aneurysm PA vasculitis Brainstem involvement Labs: ESR/APR, leukocytosis, ASCA, anti alpha-enolase Abs

Types of inflammatory dermatoses

Lichenoid (basal cell damage; interface dermatitis) -lichen planus Psoriasiform (regular epidermal hyperplasia) -psoriasis Spongiotic (intraepidermal intercellular edema) -atopic dermatitis, seborrheic dermatitis, allergic contact dermatitis Vesiculobullous (blistering w/in or beneath the epidermis) -pemphigus vulgaris, bullous pemphigoid Granulomatous (chronic granulomatous inflammation) -granuloma annulare (chronic granulomatous inflammation) Vasculopathic (pathological changes in cutaneous blood vessels) -leukocytoclastic vasculitis Panniculitis (inflammation of subcutaneous fat)

Necrotizing fasciitis

Life threatening infection of fascia just above the muscle, rapidly progressive necrosis of subQ fat and fascia Usually mixed infection affecting elderly, diabetics, alcholics, pts w/ cardiac or PVD, may have prior penetrating injury Group A strep common Initially looks like cellulits but pain is out of proportion to physical findings, area may later become anesthetic as nerves are destroyed Tx - surgical debridement, fasciotomy, amputation, IV antibiotics, supportive care, hyperbaric O2 Poor prognosis: over 50yo, underlying DM or PVD, delayed diagnosis and intervention, infection on trunk

Meissner's corpuscles

Light touch receptors found in dermal papillae Stain lightly eosinophilic, nuclei look like they are spiralling around

Juvenile dermatomyositis

Like adult disease plus: -vasculitis (esp GI) -lipodystrophy -subcutaneous calcifications

Fissure

Linear cleft through the epidermis and into the dermis

Distribution of skin lesions

Localized Regional Generalized Unilateral or bilateral Flexor or extensor Photodistribution

Pediatric scleroderma

Localized - may not need treatment or topical steroids -linear (line of scleroderma) -morphea (splotchy) Systemic -CREST -Diffuse

Pilar cyst

Look like epidermoid cysts but no punctum and do not discharge; tend to be firmer and more mobile Most on scalp Lined by stratified squamous epithelium derived from outer root sheath and demonstrating trickilemmal type of keratinization Contents are homogenous and tend to calcify

Ulcer

Loss of all of the epidermis and some of the dermis; heals w/ a scar

Chondrocytes

More round, lie in lacunae (pits) May have similar ultrastructure Important for interstitial growth

Hyaline cartilage

Most common Fetal skeleton, ribs, nose, resp tracts, and articular surfaces May or may not have a perichondrium (connective tissue covering of cartilage) Abundant translucent matrix Important in endochondral bone formation Contains chondrocytes surrounded by capsules

Scleroderma: GI

Most common system involved after skin GERD, lowered tone of LES, esophageal dysmotility Wide mouthed diverticuli or pneumocystoides intestinalis GAVE -- gastric antral vascular ectasia (watermelon stomach), due to thinning of gastric mucosa Gastroparesis Bacterial overgrowth

Exanthemous drug eruption

Most common, small erythemous papules and macules coalescing into patches and plaques Proximal >> distal (esp groin, axilla) Lots of drugs: PCNs, sulfas, cephalosporins, anticonvulsants 2 or more days after start, mainly 7-10 days, persists 2-3 days after stopping Re exposure by slow introduction may be tolerated Treatment: topical steroids, oral antihistamines, reassurance

Malignant melanoma

Most malignant tumor of skin, malignant transformation of melanocytes (either de novo (70%) or from melanocytic nevi) Early detection assures increased survival Risk factors: -genetic marker (CDKN2a) -fair skin -FHX -PHx of MM -UVR sunburns during childhood -number of nevi (over 50) and size (over 5mm) -number of atypical nevi (over 5) -atypical nevus syndrome Sx: asymptomatic, sometimes itching and ulcerated w/ bleeding Pay attention to ABCDEs 4 types: -superficial spreading melanoma (70%) -nodular melanoma -lentigo maligna melanoma -acral lentiginous

Kawasaki's

Mucocutaneous lymph node syndrome Peak age 6 mo, high mortality in acute phase Periphreal extremity changes polymorphous exanthem nonpurulent bilateral conjuctival injection Lip and oral cavity changes acute and non purulent cervical adenopathy Desquamation 1-2% die of MI soon after recovery due to coronary aneurysms Aspirin Angioplasty, thrombolytics, or CABG may be required

BCC: prognosis and treatment

30-40% will develop more BCCs Very slow growing, rarely metastasize but can be locally destructive Treatment depends on location and subtype Surgical: curette and desiccation, cryo, excision w/ 3-4mm margins, Mohs Non surgical: -imiquimod cream, 5-fluoroural cream, photodynamic therapy (reserved for superficial) -radiation (for non surgical candidates) -vismodegib (molecular inhibitor for metastatic BCC and BCNS)

ACS2: Scarlet fever

Sore throat, fever, sandpaper (fine, papular) rash in axilla and groin will peel Group B strep w/ exotoxin

Cartilage matrix

70% water 40% dry weight type II collagen Abundant GAGs (chondroitin-4 sulfate, condroitin-6 sulfate, heparan sulfate) Abundant proteoglycans

Excoriation

An erosion caused by scratching, usually linear

Abs in Sjogren's syndrome

Anti-SSA -- vasculitis, leukopenia, thrombocytopenia Anti-SSB -- associated w/ SSA [Ro]

Abs in MCTD

Anti-U1RNP Correlates w/ raynaud's, myositis, restrictive lung disease

Antibodies in systemic sclerosis

Anti-centromere Anti-topoisomerase I (anti-scl 70) Anti-RNA pol III

Apocrine sweat glands

Musk secreting glands - enters hair follicle Bacteria break down secretions to create musk Secrete by merocrine mechanism, simple exocytosis

Osteoblasts

Polyhedral, stain intensely basophilic Found on edges of bone Becomes an osteocyte once it surrounds itself with matrix that it secretes

Knee aspiration technique

Position patient (supine, towel under knee to relax quads) Find and mark site Clean and anesthetize site Aspirate

Phototherapy

-UVB (280-320 nm)/UVA (380-400 nm) to target lymphocytes in skin -Broad spectrum of conditions treated -Psoriasis, atopic dermatitis, generalized pruritus, mycosis funoides (cutaneous T cell lymphoma), others -increased risk of skin cancer due to increased UV exposure -contraindicated in autoimmune conditions w/ photosensitivity (SLE, dermatomyositis) Pretreat for UVA w/ psoralens (to increase photosensitivity)

Nutrition: osteoporosis

1500 mg/day calcium 600-800 IU/day Vit D

Nevi surveillance

ABCDEs Nevi in an individual tend to share a similar pattern May be solid brown, solid pink, eclipse (tan or pink w/ brown ring), targetoid ('fried egg') Melanoma will have a different pattern, if there is a nevus that sticks out (ugly duckling sign), biopsy that one

Churg Strauss syndrome

ALlergic granulomatosis and angiitis Sx: -severe asthma -peripheral tissue eosinophilia -extravascular granuloma formation -vasculitis of multiple organ systems -FEVERS, malaise, anorexia, weight loss -pulm: SEVERE/FREQUENT ASTHMA ATTACKS and infiltrates -mononeuritis multiplex -allergic rhinitis/sinusitis -heart disease -skin lesions -renal disease less common Labs -EOSINOPHILIA -Elevated ESR/CRP -Elevated fibrinogen -ANCA MPO

Inflammatory process

AM stiffness greater than 1 hour Improvement with activity Swelling, warmth, erythema may be suggestive Elevated inflammatory markers (ESR, CRP), synovial fluid analysis (white cell count)

Osteoarthritis: clinical

AM stiffness over 30 minutes Gel phenomenon (stiffness after inactivity) Crepitus Joint pain/tenderness worse w/ activity Bony swelling - Heberden's/Bouchard's node Functional impairment Periarticular muscle atrophy Labs will show high viscosity synovial fluid of normal clear color, w/ less than 2000 WBCs

Joint examination: soft tissue

AROM and PROM in same direction are painful, but isometric resistance is painless Inert tissue is at fault If there is pain and limitation in every direction there is a capsular pattern, sign of arthritis, affection of whole joint Non capsular pattern -ligamentous adhesions - pain in one line of motion but not others -internal derangement - intracapsular fragmets interfere with normal ROM in certain directions due to impingement, other motions are free/painless -extra-articular lesions -- disproportionate limitation, extra-articular adhesions, acutely inflamed structures Limited range of motion that is pain free: -symptomless osteoarthritis: bony end-feel If ROM is full and painless, there is no lesion of inert tissue If AROM, PROM and isometric are all painful, contractile tissue is at fault

RF

Ab that recognizes Fc portion of IgG as its Ag, produced in synovial tissue Can be positive in: -SLE -Hep C -bacterial endocarditis -parasitic infection -neoplasms -aged Associated w/ severe, erosive disease, worse functional outcomes, nodules, extrarticular manifestations

Denosumab

Ab, antiresorptive Can be used in renal failure Inhibits RANK ligand, involved in immune response Side effects: flatulence, eczema

Malignant neoplasms

Abnormal cell structure, poorly differentiated Mitotic figures common Invasive growth Rapid growth Rarely cease growing Rarely encapsulated Will metastasize if untreated Significant harm on host due to invasion and metastasis

Seronegative spondyloarthropathies

Absence of RF, association of HLA-B27 Peripheral arthritis, lower limb, asymmetric Radigraphic sacroilitis No SQ nodules or extra articular RA features Overlaping extra-articular features (anterior uveitis) Significant familial aggregation Types: Ankylosing spondylitis Reactive arthritis Arthropathy of IBD Psoriatic arthritis Undifferentiated spondylarthropathy Juvenile onset ankylosing spondylitis

Seborrheic keratosis: histology

Acanthosis (thickened epidermis) Varying degrees of papillomatosis Pseudo-horn cytst (invaginations filled w/ keratin)

Wheelchair

Acceptable for any condition, but use as a last resort. Attempt less restrictive options first

Gout: pharm

Acute attack: anti-inflammatory Colchicine - best early (until 6mg or diarrhea) NSAID (+/- indomethacin) Corticosteroids IM ACTH or IV colchicine Long term: Prophylaxis from future attacks -Xanthine oxidase inhibitors (allopurinol, febuxostat) -Uricosuric agents (probenecid, sulfinpyrasone) -pegloticase Anti-hyperuricemic

Sweet's syndrome

Acute febrile neutrophilic dermatosis Sharply marginated, rapidly extending, erythematous or violaceous, painful, elevated plaque Intensely edematous or indurated Face, neck, upper trunk, extremities Associated w/ -inflammatory and autoimmune (IBD) -underlying malignancy -drug induced -postinfectious -pregnancy related -up to 50% idiopathic

Kawasaki's disease

Acute generalized systemic vasculitis **Leading cause of acquired heart disease in children in developed countries** Self limited involving coronary arteries **present younger than 5yo ** Labs: -leukocytosis -elevated acute phase reactants -thrombocytosis -anemia -elevated liver enzymes -hypoalbuminemia

ACS2: Meningococcal infection

Acute onset (6 hrs) of fever and rash Purplish red, non blanding rash of extremities, does not blanch Dizziness, fever, high HR, low BP Gram negative diplococci

Color of skin lesions

Affected by: -color of skin -type of pigment w/in lesion -where pigment is in skin -presence or not of inflammation -thickness of epidermis -hydration of skin

Osteoporosis: risk factors

Age Glucocorticoid therapy Gender Secondary Personal history of fracture Parental history of hip fracture Femoral neck BMD Current smoking Low body mass index Alcohol

Mole (nevus)

Aggregate of melanocytes Flesh colored, pink, tan, brown, dark brown bump or spot (papule or macule) less than 3-4 mm Uniform pigment, smooth, uniform border Types: -congenital -acquired -atypical

Churg-strauss

Allergic rhinitis, nasal polyps and asthma After 2-12 yrs, attacks of fever and eosinophilia, diffuse angiitis of lungs, heart, liver, spleen, kidney, intestines and pancreas 2/3 have cutaneous lesions -nodules on extensor surfaces -firm, nontender papules on fingertips -purpura or hemorrhagic bullae -leukocytoclastic vasculitis

Pyoderma gangrenosum

Begins as inflammatory pustule w/ surrounding halo that enlarges and begins to ulcerate Sharply marginated, undermined, blue to purple borders 50% of patients have associated disease; most commonly IBD, also leukemia, myeloma, polycythemia vera, hep C, HIV, SLE, ...

Hair follicles

Begins in hypodermis in the hair bulb Bulb (1 and 2) 1 - Papillae - light staining area at base 2 - Matrix - equivalent of stratum germinativum, rich in melanocytes that pigment the hair Internal root sheath - equivalent to stratum granulosum, keratinizes the hair in a 360 degree fashion External root sheath -- comprised of multiple layers of cells, merges with epidermis, can migrate from root sheath to repopulate epidermis if damaged (or from eccrine sweat gland duct lining) Sebaceous glands -- attach to upper 1/3 of hair follicle, round nuclei, round clear cells, cells become pyknotic and die as they move toward duct (holocrine secretion -- whole (dead) cells) Erector pili muscles attached to upper portion of hair shaft

Benign hypermobility syndrome

Beighton's score (at least 4 of 9) Paint at night or going to bed awakening the child from sleep Pain mainly in legs esp legs Improved w/ rubbing, warmth, analgesics Tx: -analgesics -muscle strengthening -proper body mechanics

Telangiectasias

Benign essential telangiectasia CREST Liver disease (high estrogen levels) Pregnancy/estrogen therapy

Cherry angioma

Benign neoplasm of capillaries Clinical: -round, slightly elevated, superficial, rubbery, non blanching, bright red papules -after 30yo -most common on trunk, can be on face Treated w/ electrodessication, laser ablation or shave biopsy May bleed or thrombose mimicking melanoma

Skeletal health assessment techniques

Bio markers: -formation -- bs-alk phos, osteocalcin, PICP, PINP -Resorption: pyralink, pyrilink-D CTX, NTX Vertebral fracture assessment -at the time of BMD WHO algorithm -10 yr probability of hip fracture and any major osteoporotic fracture

Polymyositis: biopsy

Biopsy will show cytotoxic CD8 T lymphocytes surrounding and invading muscle fibers, degeneration and regeneration of muscle fibers

Blanching

Blanchable lesions -- dilated vessels, perivascular inflammation leading to dilated vessels (telangiectasia) Hemorrhage or erythrocytes in the dermis -petechia -ecchymosis -palpable purpura

Hyperuricemia

Can be asymptomatic Attack of articular and periarticular inflammation (acute gouty arthritis) Accumulation of articular, osseous, soft tissue, and cartilaginous crystalline deposits (chronic tophaceous gout) interstitial nephropathy w/ renal impairment (urate nephropathy) Uric acid nepholithiasis Most people w/ hyperuricemia never develop symptoms of uric acid excess Causes: Overproduction: ethanol, HGPRT deficiency, PRPP synthetase overactivity, chemotherapy, sickle cell anemia, myeloproliferative disorders Underexcretion: renal insufficiency, drugs/toxins (ethanol, diuretics, cyclosporine, lead nephropathy, low-dose ASA) Incidence of gout increases exponentially once a threshold serum acid level is reached

Antiphospholipid syndrome

Can be primary or secondary Must meet both clinical and lab criteria Clincal: either vascular thromboses or pregnancy morbidity Lab: -Anticardiolipin Abs - IgM and IgG -Lupus anti-coagulant -Anti-beta glycoproteins -- IgM and IgG

Reactive arthritis - clinical course

Can be progressive and chronic, polycyclic, or monocyclic Poor prognosis w/ -HLA B27+ -Male -Extra articular lesions -Post venereal disease (esp chlamydia --> majority progress to chronic arthritis)

Shave biopsy

Carcinomas, cutaneous lymphoma (MF), benign neoplasms Shave top layer with scalpel

Osteoarthritis: pathology

Cartilage degeneration at point of maximal stress, joint space narrowing Swelling, inreased proteoglycan synthesis and release of degradative enzymes --> articular cartilage thins and softens Cartilage loss --> subchondral bone sclerosis, trabecular compression, reactive bone deposition Synovial fluid forced into underlying bone --> subchondral cysts Endochondral ossification --> attempt at repair w/ osteophytes at margins IMAGE: top normal, bottom: degenerative cartilage w/ hypercellularity, low MPS

Joint examination: Physical exam

Check unaffected side first if possible AROM - active range of motion -test power, control, willingness to move PROM - passive range of motion If no limitation, apply over pressure at end of range and determine "end feel" Resisted isometric range of motion -no articular motion, muscle movement test only -assess for muscular pain and weakness -work isolated muscle group activity, rule out accessory muscles by their other functions Perform painful movements last

ACS2: Parvovirus B19

Child w/ rash, low grade fever, mild illness Bright red splotches on both cheeks and lacy rash on trunk and extremities "Slapped cheek" Complications: aplastic anemia (esp patients w/ sickle cell), miscarriage in pregnant women (contagious before development of rash)

Ankylosing spondylitis

Chronic inflammatory disease of SI joints and spine Peak onset in 20s, males over females Increased risk w/ 1st degree relative with AS Etiology unclear but HLA B27 most important predisposing factor -- most prevalent in caucasians

Acne vulgaris

Chronic inflammatory disease of pilosebaceous follicle Characterized by comedones, papules, pusultes, nodules and scarring Patients typically have variety of lesions in various stages of resolution Special forms: -steroid induced -hyperandrogenic states -lifestyle (comedogenic products (pomade) and diet)

Calcium oxalate

Chronic renal failure Bypyramidal and envelope shaped Alizarin read == Ca2+ Brightly birefringent

Papule

Circumscribed solid elevation w/ no visible fluid, diameter under 1 cm

Bulla

Circumscribed, fluid containing elevation, diameter over 1 cm

Vesicle

Circumscribed, fluid containing elevation, diameter under 1 cm

Pustule

Circumscribed, superficial collection of purulent exudate

Diagnosing gout

Classic history and presumptive triad: -acute monoarticular arthritis -hyperuricemia -gratifying response to colchicine Diagnosis is NOT: -elevated serum uric acid level -clinical presentation -response to medication Diagnosis IS: -crystal identification from synovial fluid: needle shaped, negatively birefringent, yellow when parallel to the axis of the red compensator (IMAGE) 24 hour urine collection for uric acid and creatinine -under 800mg, underexcretion, over, overproduction Synovial fluid w/ elevated WBC

Prepatellar and infrapattelar bursitis

Clergyman's and housemaid's knee Seen in coalminers, carpet layers, etc... Can be prone to infection

Lipoma

Clinical -solitary tumor of mature adipocytes -most common on trunk, can be on neck, forearms, axilla -may occur sporadically/familial or solitary/multifocal Histo: -encapsulated lobulated tumor, composed of mature adipose tissue w/ minimal fibrous septae Subtypes: -angiolipoma -spindle cell -pleomorphic -hibernoma

Sebaceous hyperplasia

Clinical: -lesions are small, on forehead, infraorbital, temples -cream to yellow umbilicated papules often w/ telangiectasias -sun exposure -can mimic early BCC or other adnexal tumors Histo: -prominent sebaceous gland lobules surrounding a dilated hair follicle

Neurofibroma

Clinical: -soft, flaccid, pink white nodule that can be invaginated by pressure w/ the finger -solitary or multifocal (3 or more consider NFM) -may be plexiform ("bag of worms") Histo: -proliferation of spindled and s shaped cells w/in dermis -stromal collagen is delicate and pale and contains mast cells Surgical excision is treatment of choice

Rocky mountain spotted fever

Clinical: painless bite w sx after 6-8 days; fever, HA, myalgias, n/v/d, abd pain Petechial eruption 2-4 days after onset of fever w/ centripetal spread Seizures, ataxia, meningitis, shock, DIC Treatment: doxycycline, chloramphenicol -treat empirically; do NOT wait for test results

Necrobiosis lipoidica diabeticorum

Collagen degernation w/ granulomatous response, thickening blood vessel walls Aysmptomatic shiny patches that slowly enlarge; patches initially red-brown and progress to yellow, depressed atrophic plaques, can ulcerate Can precede onset of DM Females > males

Crust

Collection of serum with white blood cells and/or red blood cells which has dried on the skin surface; scab

Scale

Collection of stratum corneum (dead epidermal cells); exfoliation

Lesion morphology

Color Texture Pattern Distribution

Seborrheic keratosis

Common benign superficial epidermal growth Appear in late 3rd to 4th decade -new pigmented lesion after 3rd decade should raise question of melanoma Palms and soles spared may be itchy or cosmetically bothersome Appear as greasy, stuck on papule or plaque (waxy), can vary in color and texture Gently pick or scratch at lesion, it may give way revealing the waxy character Two subtypes: -stucco keratosis - white to gray usually on dorsal feet and ankles of older fair skinned patient, often confused w/ flat warts -dermatosis papulosis nigricans -- multiple tiny SKs in darker skin types, usually on cheeks and temple Associated with adenoCAs of stomach, colon

Osteoid osteoma

Common benign tumor of any age (adolescence common) Night time pain resolved w/ NSAIDs Xray: radiolucent ovoid lesion w/ surrounding cortical thickening

Intramembranous bone formation

Common in facial bones Mesenchymal tissue gives rise to osteoblasts which will develop directly into bone

Cellulitis

Common infection Suppurative inflammation involving subQ tissue, caused most frequently by strep pyogenes or staph aureus -animal bites -- pasteurella -human bites -- eikenella Often follows a wound or tinea pedis; as most common portal of entry Spreading erythema, tenderness, infiltration, pitting w/ pressure, central part may have nodular vesicular that ruptures and discharges pus and necrotic material Common in lower extremities Malaise, chills, fever Streaks of lymphangitis may spread to draining lymph glands Complications; gangrene, metastatic abscess, grave sepsis in children/compromised Risks: local trauma, underlying skin lesion, inflammation, edema/impaired lymphatics, skin infection, secondary spread from blood or fistula Tx: empiric therapy early to prevent sepsis/death, cover both staph and strep, MRSA considered if purulent, monitor closely, treat tinea pedis if present, culture from abscess and other purulent tissue -clindamycin -TMP/SMX -doxy -linezolid -vanc

Lupus

Complex set of immunologic abnormalities w multiple mechanisms of dysregulation Immune complex disease Pathogenesis Definite: UV B light Probable: sex hormones Possible: diet, alfalfa, saturated fats, infection, toxins, drugs Drugs: -hydralazine -procainamide -isoniazid -minocycline -TNF-alpha inhibitors Drugs don't cause true lupus or flare lupus in one that has it, don't withhold drugs from those that need them (in true lupus patients)

Swan neck deformity

Contraction of the flexors of the MCPs IMAGE -- ring finger

Giant cell arteritis

Cranial or temporal arteritis Closely associated w/ polymyalgia rheumatica -stiffness, aching, pain in muscles of neck, shoulders, lower back, hips and thighs -Elevated ESR Occur in older people, more common in females, less common in dark skinned persons Sx - fevers, malaise, fatigue, anorexia, weight loss, sweats, headaches, scalp pain, tender thickened, nodular artery, jaw or tongue claudication, arthralgia **Complication if untreated: ischemic optic neuropathy ** Histo: involvment SEGMENTAL, temporal artery bx: panarteritis w/ giant cell formation (Arrows) Labs: elevated ESR, normochromic anemia, elevated LFTs, increased IgG and complements, Normal CK and other muscle enzymes

Cutaneous signs of infection: hep C

Cryoglobulinemia (leukocytoclastic vasculitis) Telangiectasias (cirrhosis) Polyarteritis nodosa Lichen planus Porphyria cutanea tarda Necrolytic acral erythema

Complications of assistive devices for ambulation

Cumulative repetitve stress injuries to upper extremities Accidents: improper device/use, equipment failure increased energy requirement Reduced walking speed (most devices)

Seborrheic keratosis: treatment

Curette -- mechanical destruction; leaves a pink, moist base Lightly freeze w/ liquid nitrogen; avoid this treatment in darker skin (melanocyte destruction) Electrodessication -- electric current to cause destruction

Seborrheic dermatitis

Dandruff Overgrowth of Pityrosporum, yest of sebaceous skin (scalp, eyebrows, central face) Infantile: first few months, affects scalp, scales and crust, ears and neck may also be involved Adult: scalp, face, neck, mid upper chest and intertriginous zones (axillae, groin, submammary, beneath pannus), eyebrows, nasolabial folds, retroauricular areas Treatment: keratolytic shampoos and gels, topical antifungals, metronidazole and/or glucocorticosteroid Difficult to treat and widespread in those w/ immunosuppression or neurologic disorders

Purine metabolism

De novo synthesis least common pathway -- PRPP synthetase rate limiting step Most purines come from salvage pathway

Scleroderma: treatment

Depends on organ system involved Renal crisis --> ACE inhibitors Interstitial lung disease - cyclophosphamide, autologous stem cell transplant PHTN -- prostacyclins, endothelin receptor antagonists, phophodiesterase inhibitors

Verruca vulgaris: treatment

Depends on type, location, age of patient, previous therapies At least 2-3 mo therapy required Destructive methods: -cryo -salicylic acid -laser -bleomycin -cantharidin Immunotherapy: -imiquimod -candida Ag -oral cimetidine (children)

Papillary layer of dermis

Dermal papillae Lots of nuclei -- belong to fibroblasts that create the matrix and collagen of the dermis

Stevens Johnson Syndrome/Toxic epidermal necrolysis

Dermatology emergency Rare, potentially fatal Path: Drugs -- sulfa, NSAIDs, allopurinol, anticonvulsants (esp carbamazepine) Infections: mycoplasma, HSV Sx: -prodrome of fever, flu like symptoms, mucosal involvement, cough, HA, v/d -erythema, blisters/erosions follow pain/burning, exxtensive necrosis and detachmetn of epidermis, exposed red, oozing dermis -Generalized LAD and systemic involvement possible Risks: slight female predominance, immunosuppression, HLA type, slow acetylator, radiotherapy w/ anticonvulsants Consult derm immediately Tx: admit to burn unit, fluid/electrolytes, d/c causative drug, ophtho consult, systemic steroids if early, IVIG 1g/kg/day for 3-4 days

Herpes zoster

Dermatomal distribution Varicella zoster virus Vesicular/bullous eruption Develops due to diminishing immunity to VZV w/ age 3 stages: -prodrome - pain, burning, stabbing, paresthesias +/- fever, fatigue, malaise -acute: vesicles, usually itchy but not in themselves painful, followed by crusts -chronic: pain after lesions have healed, lasting weeks-years afterward (postherpetic neuralgia) New lesions continue to develop for up to 1 week Hematological dissemination to other sites Management: -immunization w/ VZV vaccine -antiviral therapy -IV antivirals for immunosuppressed and ophthalmic HZV -acute pain managment (analgesics) -chronic pain management -contagious when vesicles are present in those who have not had chickenpox, immunocompromised, and in pregnancy

Condyloma acuminatum: treatment

Destructive methods: -cryo -electrocautery -excision -trichloroacetic acid -carbon dioxide laser Cytotoxic methods: -podophyllum resin or podofilox -5 FU -bleomycin -imiquimod Sinecatechins -green tea derived

Ottawa ankle rules

Determine need for radiographs after ankle injury Xray series is necessary only if there is pain on or superficial to malleolar zone AND: -bone tenderness 6cm or less of post edge or on tip of either malleolus OR -inability to bear weight both immediately and in ER (can't take 4 steps) Rules can't be applied if examiner can't palpate the bones due to excessive swelling

Joint examination: Functional assessment

Determine whether impairment affects a patients ability for ADL Activities that cause symptoms, limited by symptoms, and requirements of activities

ACS2: SJS

Development after recent administration of drug (esp antibiotics) Fever, rash, conjunctivitis, photophobia Rash is widespread and consists of macules, urticarial lesions, and some erythematous lesions w/ central vesicles "Erythema multiforme" Will usually have membrane involvement as well

Acne: pathogenesis

Exclusively a follicular disease - comedo formation by plugging of follicle w/ keratinocytes Androgen secretion, alterations in lipid metabolism, and an abnormal response to local cytokines also important After comedone, lower portion of follicle enlarged, contents discharged into dermis and inflammatory papule/pustule is formed Nodules and cysts can form as well as scars Causes: -Meds (steroids) -Propionibacterium acnes -hormones -environmental/skin products -genetics

Joint examination: accessory motion

Extension and flexion of wrist Obtained by passive movement by exam iner

Perichondrium

External covering Outer dense irregular connective tissue Inner chondrogenic layer (appositional growth -- "from the edges") Chondrogenic layer contains chondroblasts that give rise to chondrocytes Perichondrium usually not present in joints as the bones will articulate cartilage up against cartilage

Pressure ulcers: cause explains chronicity

Extrinsic pressure -ventilated, debilitated patients do not move -neuropathy, sedation impaired sensation Atrophy of dermis, collagen and fat that support microcirculation Inflammation persists -occlusion of lymphatics, venules prevents clearance of bacteria, pro inflammatory factors, necrotic tissue Proliferation does not occur; tissue hypoxia -poor perfusion and anemia limit delivery of nutrients, oxygen

Sicca

Eye -- contact intolerance, gritty sensation, redness, photophobia, ocular fatigue, AM crusty eyelids; untreated runs risk of abrasion, infection Dry mouth: water bottle sign, cracker test, accelerated tooth decay, early dental loss Susceptibility to oral candidiasis, swelling of salivary glands, risk of calculi, parotitis, abscess, can also have dry nose, xerotrachea, dry skin, vaginal dryness

SCC and BCC distribution

Face: more BCC Hand: more SCC Depth of invasion and presence of peri neural spread are important prognostic indicators

Dermatomyositis

Facial rash/heliotrope rash V sign - photosensitivity w/ rash on face and anterior chest, pruritis common, esp on scalp Holster sign -- rash on lateral thighs and hips Shawl sign -- rash over back, shoulders, proximal arm Gottron's papules (reddish purple scaling eruption over knuckles and over knees/elbows) Erythroderma Capillary loop changes in nailbed Associated w/ ovarian/GU/adenoCAs

RA: systemic symptoms

Fatigue Malaise Morning stiffness Diffuse musculoskeletal pain Low grade fever Weight loss Anorexia

ACS2: Rocky mountain spotted fever

Febrile illness w/ rash starting on wrists and ankles Rickettsia, dog tick vector, infects endothelial cells

Drug rxns: risk factors

Female Prior Hx Recurrent drug exposure w/ high risk of adverse reactions HLA type Certain disease states -EBV and ampicillin -HIV and sulfas

ACS2: Lyme disease

Fever, malaise w/ rash consisting of a red papule that spreads into a bull's eye appearing lesion, 5 inches in diameter w/ central clearing Borrelia burgdorferi -- spirochete, deer tick vector

Abscess

Firm, tender, erythematous nodule that becomes fluctuant, usually no systemic symptoms Staph aureus most common bug Causes: from primary skin infection (furuncle, carbuncle), secondarily infected skin tomor or site of skin disease or injury, direct traumatic seeding, hematogenous spread (Rare) Tx - incision and drainage, pus, culture and susceptibility testing, prophylaxis for patients at risk for endocarditis prior to I&D Antibiotics indication: -large or multiple lesions -rapid progression, surrounding cellulitis -sx of systemic infection -immunesuppression -extremes of age -difficult to drain area -lack of response to I&D alone -associated septic phlebitis

Joint examination: subjective history

First things first Review of systems Patient history Systems review -cardio/pulm -integumentary -musculoskeletal -neuromuscular -communication/cognition/affect

AS - imaging

First x ray changes in SI joints but early on may only be evident in CT or MRI Pseudo widening of SI joint early, late -- fusion of SI joints X ray -- bamboo spine -- vertebral fusion -- progressive squaring of lumbar vertebral Late stages -- calcification of anterior longitudinal ligament and anterior atlantoaxial subluxation

Crutches

For otherwise healthy/in shape persons Fracture of lower extremity (unable to bear weight on lower limb but are coordinated/strong enough to bear weight on arms)

Kyphosis

Forward curvature of spine

Basic calcium phosphate

Found in articular tissues and elsewhere -tendon -IV disc -joint capsule -cartilage -synovium Crystals difficult to detect, cannot be seen w/ LM -Aggregated crystals - "shiny coins", alizarin red stains, ID requires EM Crystals in synovial fluid from OA knee, correlates w/ severity of radiographic OA Distinctive type of destructive arthropathy -- milwaukee shoulder (IMAGE) Calcific periarthritis

Furunculosis and carbunculosis

Furuncle (boil) -- acute, round, tender, circumscribed perifollicular staph abscess generally ends in central suppuration Carbuncle -- two or more confluent furuncles w/ separate heads Occur in hair bearing skin Risk fx: warm, humid environment, obesity, hyperhidrosis, maceration, friction, dermatitis, DM, staph aureus carriage, malnutrition, HIV infection or other immune suppression Tx: culture, small furuncle treated w/ warm moist compression, larger managed like an abscess, systemic drugs usually not needed, but commonly treated w/ oral antibiotics

Musculoskeletal exam

GALS -- gait, arms, legs, spine Any pain or stiffness? Can you dress yourself? Can you walk up and down stairs? Gait -- symmety, smoothness, stride length, strike, stance, toe-off, swing, turning Inspection from all sides and during spine flexion Check movements of arms, hands, fingers

Large vessel arteritis

Giant cell arteritis Takayasu's arteritis

Myopathies: treatment

Glucocorticoids -most patients have some response -fasted acting -start high, taper as soon as possible to lowest dose -GC myopathy can confound assessment Immunosuppresive medications Intravenous gamma globulin Topical treatments Physical therapy -passive stretching to prevent contractures -isotonic, isometric exercise, strength training later Rash of DM: HCQ, quinacrine, isotretinoin, topical tacrolimus Calcinosis: no real effective treatment, colchicine, warfarin, alum OH, probenecid, diltiazem, bisphosphonates

GPA (Wegener's)

Granulomatous vasculitis of -upper and lower resp tracts -glomerulonephritis -can involve any organ system Middle aged caucasians Sx - upper resp tract (sinus pain w/ drainage, nasal mucosal ulceration), nasal septal perforation, eustacian tube blockage, subglottic tracheal stenosis, pulmonary (present in 90%), eye involvement, skin lesions Cardiac involvement: pericarditis, coronary vasculitis, cardiomyopathy CNS: mononeuritis multiplex, cranial neuritis, cerebral vasculitis Renal disease Labs: elevated ESR, mild anemia, mild hypergammaglobulinemia (IgA), elevated RF, thrombocytosis **ANCA in 90% -- proteinase 3**

Erosive OA

Gull wing deformity (2nd DIP on image) Periarticular osteopenia not present

Condyloma acuminatum

HPV warts ** most common STI** Single or multiple papules, may be verrucous, pearly, couliflower like or plaque like, can be smooth (particularly on penile shaft) Most common on: -penile glans and shaft -vulvovaginal and cervical -perianal ID of condyloma lesions should prompt search for evidence of other STDs Related to HPV types 6 and 11 (least likely to cause cancer), 16 and 18 most likely to cause cancer

OA: joint involvement

Hands: distal IPs, proximal IPs, first metacarpal (thumb) acromioclavicular in shoulder C/T/LS spine Hips -- cysts in superior acetabulum Knees First metatarsalphalangeal

Special skin lesions

Have excdptionally peculiar structure Have odd mechanisms of production Limited to a few dermatoses Burrow Petechiae Purpura Lichenification Telangiectasia Ecchymoses

Haversian system/osteons

Haversian canal -- where blood vessels and lymphatics are found Surrounded by lamellae of bone, osteocytes communicate to each other through canaliculi to exchange nutrients Partial osteons evidence of active bone remodeling Volkmanns canal -- two haversion canals connected to each other without distinct circular lamellae around them

Non ANCA small vessel vasculitides

Henoch schonlein purpura Essential mixed cryoglobulinemia Behcet's syndrome

Idiopathic inflammatory myopathies

Heterogenous group of disorders characterized by chronic inflammation of striated muscle and skin Painless proximal muscle weakness (except inclusion body myositis) Onset usually insidious, progressive but can be variable or acute in presentation Bimodal age of onset (late childhood and middle age), twice as likely in women as men (exception inclusion body), more common in african americans than caucasians than japanese

ESR abnormalities

High ESR: Infection Connective tissue disease, vasculitis, inflammatory arthritis Cancer Anemia Aging Pregnancy Many more LOW ESR: Afibrinogenemia/dysfibrinogenemia Aggamablobulinemia Extreme polycythemia Increased plasma viscosity

Impetigo

Highly contagious, superficial infection caused by staph aureus, strep. pyogenes or both Non bullous: papules w/ erythema, pustules break down to honey colored crust (usually staph) Bullous: affect children mostly, flaccid bullae, face and hands, caused by coagulase positive staph Ecthyma: ulcerative staph or strep pyoderma, shins or dorsal feet, begins as vesicle, vesicopustule, enlarges, saucer shaped ulcer w/ a raw base and elevated edges Tx - oral antibiotics (dicloxacillin, cephalexin, erythromycin, clindamycin, amoxicillin), topical mupirocin ointment in healthy patient w/ local disease

Histology of bone

Highly eosinophilic Trabeculated Bone marrow spaces in between eosinophilic trabeculae -- contains abundant unilocular fat (yellow marrow) or no fat (red marrow)

Basal cell carcinoma

Histo: rounded nests of "basaloid" cells, peripheral palisading, fibromyxoid stroma, cleft formation Tumor derived from basal keratinocytes Most common skin malignancy, most patients caucasian Risks: UV exposure, ionizing radiaiton, genetics, immunosuppression Secondary to PTCH (TSG -- sonic hedgehog pathway) mutation -acquired (UV damage) or inherited Classically appear pearly, pink, papules w/ a rolled border and overlying telangiectasias Frequently ulcerate and bleed

Acanthosis nigricans

Hyperpigmented, velvety plaques Affect the face, neck, axilla, groin May be associated w/ -malignancy -obesity/insulin resistance/DM -hereditary

Nutrition and healing

Hypoalbuminemia leads to edema -reduces tissue tolerance for pressure -decreased oxygenation Non nutritional factors such as trauma, sepsis, chronic inflammation -may lower serum albumin -further increase risk for tissue breakdown Challenges: Wound bed in catabolic state Increased catabolic hormones Increased cytokines Increased oxidants Increased catecholamines Decreased endogenous anabolic activity

Gout stages

I - asymptomatic hyperuricemia II - acute intermittent gout/acute gouty arthritis III - intercritical gout IV - chronic tophaceous gout

Enteropathic arthritis

IBD - Crohn's and UC - different dz but similar arthritis Axial, similar to AS, symptoms of spinal involvement do not vary w/ intestinal disease activity Highly associated w/ +HLA-B27 Peripheral joint - MCP, PIP, knees, ankles, more often in crohn's than UC, typically non destructive, reversible but can get erosions Symptoms coincide w/ gut activity, in UC remission may coincide w/ total colectomy, but some arthritis can begin post surgery Extra manifestations -clubbing -uveitis (can be bilateral and chronic) -skin - pyoderma gangrenosum Treatment: -sulfasalazine -azathioprine -steroids -methotrexate -anti-TNF May also include -bypass arthritis -celiac disease -whipple's -collagenous colitis

Joint examination: palpation

Identify tissue at fault before attempting to palpate for tenderness -eval unaffected side first -area must be in a relaxed state -can cause major pain Determine: -difference in tissue tension and texture -effusions, spasms, tonicity -fiber direction and fibrous bands Tenderness, temperature (heat if active/acute lesion), edema, swelling, pus, bony or hard swelling, pulses, tremors, dryness or excessive moister, crepitus

Legg-Calve-Perthes Disease

Idiopathic avascular necrosis of femoral head Young children Boys > girls Mostly unilateral Sx: limp, hip pain Sign: decreased ROM of hip secondary to pain Xray: initially normal, over time, disintigration of head of femur (IMAGE -- right side)

Lichen planus

Idiopathic inflammatory disorder Papules are flat and surmounted by subtle fine white dots and lines that resemble lichen (Wickham striae) More common in women Extremely pruritic and may complain of erosions if membranes involved, nail may be involved (ridging, splitting, pterygium formation) Primary lesion (4Ps): purple, polygonal, pruritic, flat papule Can be secondary to trauma from scratching Wrists and ankle favored, can be localized or widespread May heal w/ residual hyperpigmentation Rule out lichenoid drug eruption and GVHD Lab: biopsy, Hep C tests Treatment: steroids, retinoids, light therapy, cyclosporine Variable course, Tcell immune mediated?

Immediate vs. delayed reaction

Immediate - less than 1 hour of admin -urticaria, andioedema, anaphylaxis Delayed - after one hour, usually more than 6, can be days, even weeks to months after start -exanthemous eruption -fixed drug eruption -systemic reaction (DIHS, SJS , TEN) -vasculitis Onset of rash marked as day 0

Myositis and cancer

Increase relative risk for malignancy in patients w/ myositis Great majority are evident at time of diagnosis or 1-3 years after Routine test abns should be vigorously persued Common malignancies should be excluded: -PAP, pelvic exam, mamogram, CXR, colonoscopy, prostate exam, PSA, -Beware weight loss!

AS - prognosis

Increased severity - -hip arthritis -high ESR -poor efficacy of NSAID -limitation of ROM of L spine -sausage digits -oligoarthritis 0onset less than 16yo

Arthocentesis: indications and contraindications

Ind: Almost always A joint effusion that has never been tapped Contra: Established overlying infection (known cellulitis) If joint inflammation is thought to be cause of infection, then centesis should be persued Don't puncture disrupted skin Risks Bleeding, infection, damage to organs, pain

Rete ridges and dermal papillae

Indicates thick skin -- skin exposed to lots of shearing stresses Bottom of foot for example

ACS2: Roseola

Infants w/ three days of fever which breaks and is followed by rash Fine, macular, erythematous rash concentrated on trunk Herpes virus 6 is causative agent

Dermatophytoses

Infection by dermatophytes that infect only dead keratinocytes (stratum corneum, hair, nails) Three genera of fungi: -microsporum -trichophyton -epidermophyton Tinea: corporis - body pedis - feet manuum - hands capitis - head barbae - beard area cruris - groin unguium - nails

Herpes simplex virus

Infection w/ HSV-1 is one of most prevalent worldwide Infections are classified as either first episode or recurrent Most frequent manifestation "cold sore" or "fever blister" Lips near vermillion border most frequent location Grouped vesicles on erythematous base: "rain drops on rose petals", prodrome of tingling or burning UV light and stress frequent triggers, may cause infection

Causes of monoarthritis

Infectious Inflammatory -crystal induced -RA -extended oligoarticular polyarticular - JIA -Seronegative spondylarthropathies -psoriatic arthropathy -foreign body synovitis -sarcoidosis Non-inflammatory -osteoarthritis -internal derangement -osteonecrosis -synovial osteochondromatosis -reflex sympathetic dystrophy -hemarthrosis -neuropathic -stress fracture -transient regional osteoporosis -juvenile osetochondroses Tumors -pigmented villonodular synovitis -lipoma arborescens -synovial metastasis from solid tumors -synovial sarcoma

Causes of a red hot swollen joint

Infectious (bacterial, neisserial, mycobacteria, viral, lyme disease) Crystal induced (gout, CPPD - pseudogout, hydroxyapatite) Trauma/fracture Palindromic rheumatism Psoriatic arthritis Bacterial endocarditis Sarcoidosis

Scabies

Infestation by a mite, spread by skin to skin contact or contact to bedding/clothing w/ live mites Itch caused by sensitization to mite, which occurs 3-4, intense itching Lesions: -spares head and neck -burrows with minute vesicle or papule at the end of the tunnel -scaling and crusting in norwegian scabies Nodules on penis/scrotum pathognomonic for scabies until proven otherwise Management: -topical scabicides -oral ivermectin -precipiated sulfur -- pregnant women -treat infected individuals and close contacts simultaneously -wash bedding, clothing, towels or remove from contact more than 3 days -post scabetic itching persists to several weeks post treatment -contagious

Stages of wound healing

Inflammation -- 1-4 days, neutrophils, collagen III Proliferation -- fibroblasts and macrophages, cleaning up wound, epidermal proliferation, laying down collagen, remodeling, collagen I Maturation -- decrease in all cell types, remodeling of deposited collagen

Acute intermittent gout/acute gouty arthritis

Initial episode usually follows decades of asymptomatic hyperuricemia Rapid development of warmth, swelling, erythema, severe pain in affected joint First attack usually monoarticular in first MTP May have fever, chills, malaise Untreated course varies, mild pain for hours to severe arthritis for up to two weeks Early: infrequent episodes of arthritis w/ long intervals between attacks Late: attacks become more frequent, longer in duration, and involve more joints Intercritical periods free of symptoms

Myopathy: workup labs

Labs - CK, aldolase, LDH -ALT and AST can be released from injured skeletal muscle -aldolase can be elevated in hepatic disease or with hemolysis CK can be normal or low in late disease, IBM, and cancer related myositis Elevated CK does not indicate active inflammation CK can be "elevated" in african americans or other ethnicities, also trauma, exercise, seizures, etc CK is not elevated in steroid induced myopathy

Incisional biopsy

Large lesion, representative sample

PTH

Injection Intermittent use has positive effect on bones as opposed to continuous exposure of hyperPTH

Vesiculobullous pattern

Intraepithelial or sub-epithelial blistering Level of separation in skin correlates w/ pathogenesis of disease Intraepidermal: pemphigus vulgaris Subepidermal: bullous pemphigoid

Psoriasis

Intralesional injections w/ steroids Topical steroids and calipotriol Retinoids: acetretin, tazarotene Immunomodulators: MTX, cyclosporine Biologics Phototherapy

Contact dermatitis

Irritant and allergic types Irritant: chemical exposure (acute corrosion, acute irritation, cumulative irritation and phototoxicity) -presentation variable, onset related to exposure -treatment: barrier protection, irritant avoidance, petroleum jelly, NSAIDs, topical antihistamines or glucocorticosteroids Allergic: type IV hypersensitivity rxn, erythematous, scaling, papulovesicular dermatitis at site of contact, diagnosis by patch testing, treatment is allergen avoidance and topical glucocorticosteroids and oral antihistamines, prednisone

Patch

Larger macules, nonpalpable, circumscribed, change in skin color, diameter over 1 cm

Plaque

Larger, plateau-like elevation that occupies a greater surface area than its height above the skin, diameter over 1 cm

Derm: cosmetic therapy

Laser: vascular/pigmented/photoaging/tattoo/scar treatment Botox: rhytides Fillers: photoaging, AIDS associated lipodystrophy Liposuction Vein therapy

Scleroderma: musculoskeletal

Joint contractures: wrists, hands, elbows tendon friction rubs Myopathy/myositis, mild proximal muscle weakness, elevated CK Osteolysis, bone resorption of digital tufts

JIA

Juvenile idiopathic arthritis Most common rheumatic disease of childhood Children less than 16yo Persistent arthritis in one or more joints for 6 or more weeks Exclude other diseases Classifications: Oligoarthritis (persistent or extended) Polyarthrithis, RF+ Polyarthritis, RF- Systemic onset Psoriatic arthritis Enthesitis related Undifferentiated

Microscopic derm tests

KOH -- tinea: hyphae in skin/hair Scabies prep : mite, eggs, waste Trichogram -- hair abns: genetic and acquired Tzanck smear - HSV/VZV evaluation: can't tell apart Biopsy -H&E slides (shave, punch, incisional, excisional, wedge biopsies) -Direct immunofluorescence (concern for immunobullous, CVD or vasculitic process)

Pannicullitis

Key inflammatory pattern: inflammation of subcutaneous tissue Can be septal or lobular or mixed Most are mixed or lobular

Warning signs for lymphoma

LE purpura Increased salivary size w/ dominant mass Lymphadenopathy Splenomegaly Pulmonary infiltrates New leukopenia, anemia Loss of previous positive autoantibody Low C4 Cryoglobulins

Autoantibodies

Markers of chronic immunoinflammation and rheumatic disease Valuable tools for evaluation of patient w/ suspected rheumatic disease in the appropriate clinical setting

Tumor

Mass that is generally larger than 2cm, of variable depth, shape and consistency Usually refers to a neoplasm -- but no inherent meaning of malignancy

Osteocyte

Mature cell in bone matrix Stellate in shape Lighter staining

Mohs

Maximal conservation of tissue Low recurrence rate Indications: -location: nose ears, lips, scalp -aggressive histology: infiltrative, morpheaform, micronodular -large tumors or tumors w/ indistinct borders -recurrent tumors

Atypical ANCA

May combine features of cANCA and pANCA patterns w/ faint diffuse cytoplasmic staining and strong linear perinuclear staining May be present from reactivities to other nuclear, cytosolic and granular autoantigens -anti-ribosomal Ab -smooth muscle Ab and anti-actin -polyclonal hypergammaglobulinemia

ACS2: Rubeola

Measles -- paramyxovirus Maculopapular rash that begins on face and spreads to the trunk Deep red but blanching Pneumonia w/ conjunctivitis, mucous membrane involvement (white spots) MMR --> Immigrant patient would not have been vaccinated

ESR

Measurement of distance in mm that RBCs fall in tube over 1 hour Indirect measurement of acute phase reactants and quantitative immunoglobulins Anything leading to rouleax formation, thus will increase the rate of falling, and higher ESR Normal: Children less than 5mm/hr Adult less than 15mm/hr Affected by multiple variables and takes longer to decrease (not an acute indicator)

Polyarteritis nodosa (PAN)

Medium vessel vasculitis associated w/ HBV, HCV, IVD, SLE, IBD Most dianostic lesion: subQ nodules along coase of vessels overlying skin is normal or slightly erythematous, can ulcerate Livedo reticularis AND subQ nodules strongly suggests PAN

Lentiginous nevus (dysplastic/Clark's nevus)

Melanocytic nests in dermis and epidermis (compound nevus)

pANCA

Microscopic polyangitis perinuclear ANCA Zone of higher intensity surrounding the nuclear lobe w/ almost negative nuclear center Methanol fixed is usually negative, ethanol positive Myeloperoxidase Ab

Reactive arthritis - labs

Mild noromochromic, normocytic anemia Elevated ESR and CRP, complements RF and ANA negative Inflammatory synovial fluid Look for infection -- stool culture, GU swab, urine culture, but may be negative by time of appearance of arthritis Imaging - enthesitis, bone spurs (bony outgrowth dud to enthesitis)

4 Wheeled walker

Minimal effort to propel it, for patients that need stability and offloading and energy conservation Many have a seat so patient can stop and rest if necessary Ex: severe mitral regurg w/ exhaustion, end stage COPD, PHTN, CHF w severe dyspnea/SOB on exertion

Lupus: clinical

More common in females, ethnic origin affects prevalence Classification criteria: Anti-dsDNA is the trump card --> think lupus When associated w/ renal disease -- certainty of diagnosis is over 90% -malar rash -discoid rash (IMAGE) -photosensitivity -other skin findings (raynaud's, rash on head, limbs, trunk) -Oral ulcers (usually painless unless infected) -arthritis (nonerosive, involving 2 or more peripheral joints w/ tenderness, swelling or effusion, most severe is Jaccoud's arthropathy) -serositis (pleuritis or pericarditis) -renal disease (Persistent proteinuria or cellular casts, RBC, Hb, granular, tubular or mixed) -neurologic disease -hematologic (hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia) -immunologic (anti-dsDNA, anti-sm, antiphospholipid Abs) -ANA

JIA: Polyarthritis, RF-

More common than RF+, Symmetrical arthritis in >= 5 joints Large and small joints (knees, wrists, MCPs, PIPs, DIPs, ankles, MTPs, elbows, hips, C spine, TMJ, shoulders) Females > males Onset at any age Low grade fever, mild anemia, malaise, iridocyclitis rare 10% severe arthritis

RA: medical treatment

NSAIDS - potential for liver, kidney, GI tox Corticosteroids - many side effects Disease modifying anti-rheumatic drugs (DMARDS) - liver, BM tox (methotrexate, sulfasalazine, gold sodium thiomalate, plaquenil, cyclosporine, leflunomide) Biologics - cytokine blockade that drastically change outcomes and morbidity of RA, potential for reactivation of latent TB, infections -subq or infusions, aimed at TNF-alpha, CD20, IL-1, CTLA4, IL-6, JAK (oral) -Anti-TNFs most common

JIA: treatment

NSAIDs DMARDs -methotrexate -HCQ -sulfasalazine -arava Corticosteroids -systemic or intraarticular Biologics Physical therapy, occupational therapy, patient/family education

Reactive arthritis - treatment

NSAIDs Local steroid injections Skin lesions -- MTX (methotrexate), retinoids, phototherapy Eye - topical steroids, mydriatics, cycloplegics SSZ (sulfasalazine) for peripheral arthritis TNF blockers

Burrow

Narrow, elevated tortuous channel produced by a parasite

Wegener's

Necrotizing granulomas of the upper and lower resp tract, generalized necrotizing angiitis of the medium sized blood vessels Present w/ rhinorrhea, severe sinusitis, ulcerations in upper airways/nasal mucosa Systemic malaise: fever, weight loss chills Parenchymal lung involvement "Strawberry gums" Nodules appearing in crops, esp along extensor surfaces of arms Firm, tender, flesh colored to violaceous nodules may ulcerate Necrotising angiitis of skin may become evident as purpura in form of petechial or hemorrhagic pustular eruption

Microscopic polyangiitis

Necrotizing vasculitis w/ few or no immune complexes Renal involvement very common Pulm less common Renal biopsy identical to Wegener's Histo: similar to PAN except small-medium vessel involvement as well as capillaries and venules Older adult males more than females ***Similar features to Wegener's*** Labs: ESR/APR Anemia Leukocytosis Thrombocytosis **ANCA: MPO**

Neonatal lupus syndromes

Neonatal lupus rash lasts average of 17 weeks Hematologic or hepatic abnormalities less common Other organ systems of NLS usually disappear w/ the clearance of maternal Abs (6-8 months postnatal) anti-SSA or anti-SSB places fetus at high risk for development of congenital heart block, risk increases to 18-20% if previous child affected If CHB is present w/o structural abnormalities, mother is likely anti-SSA or anti-SSB positive even if asymptomatic

3 Wheeled walker

Never medically indicated for any condition NOT STABLE

Standard walker

No wheels For bilateral leg injury/weakness, need good upper extremity strength

Macule

Nonpalpable, circumscribed, change in skin color Diameter less than one cm Usually confined to epidermis

AS - labs

Nonspecific 95% are HLA B27+ Many have elevated ESR Possible increase in alk phos

End feels

Normal: -firm (capsular) -- hardis arrest of movement w/ some give to it -soft tissue end feel - no further range available due to engagement of another body part; passive flexion of knee or elbow -bony (hard) -- abrupt halt to motion when 2 hard surfaces meet, passive extension of elbow Abnormal -bony (hard) no further passive motion possible -soft tissue approximation (pathologigal if due to obesity or swelling) -firm (capsular) -- pathological if felt prior to normal full ROM; indicates chronic "arthritis" -Spasm -- "twanging of muscle" , indicates acute or subacute "arthritis" -springy block -- intra-articular displacement or internal derangement -empty -- pain experienced before extreme of ROM is reached, no organic resistance to the motion; indicative of serious pathology

Psoriasiform pattern

Path: regular epidermal hyperplasia Ex: Psoriasis Histo: hyperkeratosis and parakeratosis, neutrophils in stratum corneum (munros) and spinosum (spongiform pustule), regular epidermal acanthosis (thickening), dilated capillary loops, dermal inflammatory infiltrate of lymphocytes and neutrophils Inflammatory, increased epidermal proliferation and accumulation of stratum corneum Onset usually early adulthood and gradual, but can be variable Chronic, recurrent, well demarcated, pink plaques covered w/ silvery scales (removal of scale results in pinpoint bleeding) Prediliction for scalp, nails (pitting, onycholysis, oil spots), sacrum and extensor surfaces of limbs (tend to be symmetric) May have itching Lithium, b blockers, NSAIDS may worsen it Severe form associated w/ AIDS Therapy: topical steroids, topical tars and antrhalin, phototherapy, MTX, retinoids, cyclosporine, biologics TH1 mediated --> cytokines and TNF

Lichenoid pattern

Pathology: basal cell damage; interface/lichenoid dermatitis Ex: lichen planus

Granulomatous pattern

Pathology: chronic granulomatous inflammation Ex: granuloma annulare -- dermal collection of histiocytes and lymphocytes surrounding areas of altered collagen (necrobiosis) Mucin deposition and intradermal histiocytes Idiopathic inflammatory disorder, more common in women, can present at any age, most resolve spontaneously Can be localized or generalized: Localized (most common) -- pink/purple thinly bordereed annular papules and plaques, appear on lateral or dorsal surfaces of hands, elbows, dorsal feet, and ankles, leave no residua when resolved Generalized -- diffuse symmetric, papular or annular eruption of 10-100s of lesions, favor neck, upper trunk, upper extremities, asymptomatic or pruritis, may be associated w/ DM Treatment: topical steroid for localized lesions

AS - treatment

Patient education and regular exercise Physical therapy w/ supervised exercises preferred over home exercises Patient associations and self help groups may be useful Extra-articular -- managed by other specialits, be aware of increased risk for CV disease and osteoporosis NSAIDS, including coxibs recommended as first line for pain and stiffness, remember CV, GI and renal risks of NSAIDS Analgesics Glucocorticoids - local injections, not systemic steroids Anti-rheumatic drugs -sulfasalazine for patients w/ peripheral disease -anti TNF therapy for patients w/ persistently high disease activity despite other treatment -second TNF blocker if first stops working Surgery -total hip arthroplasty for those w/ refractory pain, disability or structural damage -spinal corrective osteotomy for severe deformity -vertebral fractures

Septic arthrtitis of hip

Patient more ill/toxic appearing Refusal to walk/bear weight Second most commonly involved joint after knee Staph/strep infection Tx: antibiotics, joint drainage and lavage

Reed's syndrome

Patient w/ multiple leiomyomas (smooth muscle tumors) -pseudo-darier sign may be present -- transient piloerection or elevation of lesion induced by rubbing -family history -uterine lesions -renal cancer Genetic defect in gene coding fumarate hydratase on chromosome 1

Arrangement/configuration of skin lesions

Pattern or random distribution -linear -follicular/perifollicular -annular (ring shaped w/ central clearing) -arcuate -grouped (coalescing lesions) -zosteriform (following dermatomes) -reticulate (lace/net like) -nummular (coin shaped)

CRP

Pentamer Can activate classical complement pathway Produced as acute phase reactant by liver in response to IL-6 and other cytokines Rises w/in 4 hours of tissue injury, peaks over 1-3 days, can fall rapidly Marked CRP (over 8-10mg/dL) may suggest bacterial infection, vasculitis, malignancy Not a good indicator for lupus activity

Intercritical (interval) gout

Periods between gouty attacks -- usually asymptomatic Some patients never have a second attack, most occur w/in 6mo - 2yrs Frequency of attacks usually increases in untreated patients

Rosacea

Persistent erythema of the face w/ telangiectasias and flushing/blushing -can occur in response to various stimuli -cheeks and nose most commonly affected Acneiform papules/pustules -comedones not present Ocular findings include conjunctivitis, keratitis, iritis -may complain of gritty, burning or stinging sensation in eye Rhinophyma - cobblestoned red plaques to cauliflower like growth of nose

Derm and drug reactions

Photo-toxic Photo-allergic Systemic hypersensitivity rxns -Drug rash w/ hypersensitivity and systemic symptoms Stevens-Johnson syndrome Toxic epidermal necrolysis

Photo drug reaction

Photodistributed rash secondary to drug **Phototoxic: can occur in any patient -antibiotics: tetracycline, doxycycline, cipro -looks like sunburn -no previous exposure required **Photoallergic: occurs in patients w/ an allergic reaction -HCTZ, amiodarone -looks more like a rash -REQUIRES previous exposure

Petechia

Pinpoint to round, red spot resulting from intradermal hemorrhage; non palpable, non blanching, less than 1 cm diameter, "red macule"

RA: cytokines

Pro inflammatory: TNF alpha, IL-1 Anti-inflammatory: soluble TNF receptor, IL-10, IL-1 receptor antagonist

Dermatophytoses - pathogenesis

Produce keratinases that allow invasion into keratinized tissue Three modes of transmission: -human/human -animal/human -soil/human or animal

Melanocytes

Produce melanin in melanosomes Found in stratum germinativum -- can see melanin deposition (brown/black)

Osteoarthritis

Progressive degenerative disorder that affects joints of hands, spine and those that bear weight No systemic symptoms, mild inflammation if at all Risk factors: obesity, heredity, age, previous joint trauma, smoking, certain occupations

Pressure ulcers: treatment

Protect wound and usrrounding skin from further trauma Promote clean wound bed, prevent infection Maintain moist wound environment Control exudate Eliminate dead space Diagnose and treat infection Surgical repair

Osteomyelitis of hip

Proximal femur most common site in children Sx similar to septic hip but child will allow gentle manipulation of the hip joint Bone edema and hyperintensity on MRI (not seen in septic arthritis)

Diffuse systemic sclerosis

Proximal skin thickening of trunk, upper arms, thighs, symmetrical involvement of fingers, hands, arms, face/neck Rapid onset following raynaud's onset Significant visceral disease - lungs, heart, GI, kidneys absence of anti-centromere antibodies Typically poor prognosis

Scleroderma: skin changes

Puffy hands, followed by thickening of skin distally and progressing proximally Pruritus common Diffuse hyperpigmentation, may change to spotty hypopigmentation (salt and pepper appearance) Telangiectasias Decreased oral aperture "neck sign" ridging and tightening of neck on extension Calcinosis Digital ulcers

Henoch-schonlein purpura

Purpura, arthralgias, abd pain, renal disease Mottle purpura of extensor surfaces, hemorrhagic w/in a day, fade in 5 days Primarily male children, triggered by viral infection or strep pharyngitis Foods, drugs, lymphoma, other bacteria IgA deposition around vessels

Pressure ulcers: risk factors

Quadriplegics Neurosurgery Post op ortho patients Critical care patients Prolonged anesthesia time Debilitated AND over age 70

Offset cane

Question mark cane, single point of contact on ground Also good for adding another point for proprioception but ergonomic shape provides for better stability Ex: mild unilateral non painful limb weakness, to offset weight onto other side, mild/moderate unilateral pain

Molluscum contagiosum

Self limiting epidermal viral infection occuring in children and sexually active adults Skin to skin contact, can have autoinoculation In adults occur in genital regions **Persists months to 5yrs then resolves, HIV infected patients have hundreds of lesions generally affecting face, observation is an option** Sx: none or mild pruritis** Skin lesions **flesh colored dome shaped umbilicated papules or nodules*** Treatment: **destruction w/ curettage,c ryo** **Topical tretinoin cream** Contagious -- "bed and bath" require close contact

Bacterial endocarditis

Septic emboli Janeway lesions - non tender purpuric macules, usually on palms and soles Roth spots -retinal hemorrhages w/ pale or yellow centers Osler's nodes (immune complexes) -tender purpuric lesions on pads of the fingers/toes

Anti-synthetase syndrome

Severe muscle involvement -- poor treatment response Fever ILD Arthritis Raynaud's Mechanic's hands

Drug reactions

Simple exanthemas and urticaria comprise majority Common w/ aminopenicilliins, sulfas, NSAIDs Immune status (HIV, EBV, HHV6, CMV) and genetics (metabolism) pre dispose Inherent chemical structure (allergenicity), dosage are other factors Get complete drug history from patient

RA: extra articular manifestations

Skin -nodules -vasculitis -fragility -pyoderma gangrenosum Eye -scleritis -episcleritis -iritis -keratoconjunctivitis -sicca -scleromalacia perforans (IMAGE) Pulmonary -pleural effusions -interstitial lung disease -bronchiolitis obliterans -nodules -vaculitis Cardiac -pericarditis -pericardial effusion -vasculitis -valve disease -conduction defects -CAD Bone -Osteoporosis -osteopenia Neuro: -entrapment neuropathy -cervical myelopathy -peripheral neuropathy Heme -anemia -thrombocytosis -lympadenopathy -Felty's syndrome (RA, splenomegaly, neutropenia) Kidney -amyloidosis -vasculitis

Hyperthryroidism

Skin is warm, moist, smooth Hair is thin Nail changes Hyperhidrosis Pretibial myxedema Grave's disase: exopthalmos

Slipped Capital femoral epiphysis

Slippage of femoral head posteriorly Limp, abnl internal rotation of hip Early adolescence Male > female Obese child or tall thin child w/ recent growth spurt Urgent orthropedic referral

Kaposi's sarcoma

Slow growing blue red plaques/nodules HHV-8 4 subtypes: -chronic/classic (feet, Med descent) -african endemic -iatrogenically immunocompromised patients -AIDS related

RA: joint involvement

Small joints in hands and feet (MCPs, MTPs, PIPs) symmetric -ulnar styloid prominence -ulnar deviations - subluxation of MCPs -fusiform swelling (IMAGE) -boutonniere deformity -swan neck deformity -cock up or hammer toe deformity -flattening of arch -hind foot valgus deformity Spares DIPs Large joints involved later Spares thoracic and lumbar spine

Hereditary hemorrhagic telangiectasia

Small tufts of dilated capillaries scattered over mucous membranes of the skin Nose bleed, melena, GI bleeding, splenomegaly, pulm and CNS AV fistulae, rarely renal aneurysms Telangiectasia AD disorder Germline mutation in endoglin or ALK-1 genes

Henoch-Schonlein purpura

Small vessel Childhood Peak incidence in spring Immune complex deposition disease w/ IgA Palpable purpura on buttocks and lower extremities, polyarthralgia but rarely frank arthritis GI involvement in 70% of peds patients -colicky -n/v/d -constipation w/ blood per rectum -intussusceptions Renal involvement: -glomerulonephritis -renal involvements in adults more insidious and severe

RA: radiographics

Soft tissue swelling Periarticular osteoporosis Symmetric joint space narrowing Marginal erosions Deformities, malalighnment MRI-flexor/extensor tenosynovitis, bone marrow edema, erosions

Types of crepitus

Soft/fine - minor roughening of articular cartilage Coarse -- superficial fragmentation Creaking -- pathology in tendons Snapping -- tendon moving over bony prominence

Fixed drug rxn

Solitary erythematous patch which recurs at same site w/ reexposure Often on oral and genital mucosa, face and acral areas May occur from 30 minutes to 8 hours after ingesting the drug Red patch --> iris/target lesio; may blister and erode Prolonged or permanent hyperpigmentation May have multiple lesions (generalized fixed drug eruption) Associations w/ : -NSAIDs, sulfas, TMP, barbiturates, tetracyclines, salicylates, food coloring, celecoxib, herbals -HLA B22 -pseudoephedrine (non pigmenting variant) -Baboon syndrome: buttocks, groin, axillae (aminopenicillin or cephalosporin, contrast media) Tx: resolve days to weeks after discontinuation, topical steroids for non eroded lesions, antibacterial ointment for erosions, if generalized derm referral

Myotoxicity and lipid lowering drugs

Statins, fibrates, niacin Effects variable +/- muscle symptoms, +/- rise in CK Myalgias, muscle tenderness or weakness Aching, cramping esp in trunk and prox muscles Tendon pain Nocturnal leg cramps Difficulty w/ grip Symptoms develop average of 6mo into therapy and can continue for months after withdrawal Genetic predisposition to statin myopathy Anti HMGCR ab demonstrating an immune mediated etiology of statin induced myopathy in some cases

Joint examination: isometric results

Strong and pain free -- no contractile or neural lesion Strong and painful -1st or second degree muscle damage Weak and pain free -3rd degree strain, rupture of a muscle -peripheral nerve or nerve root involvement Weak and painful Severe lesion: resistance leads to weakness and increased pain, major strain but not full rupture or fracture All movements are painful -pain is result of fatigue, emotional hypersensitivity or emotional problems -attempt resisting movements remote from site of pain

Features suggesting autoimmune myopathy

Subacute symmetrical proximal weakness Family history of autoimmune disease Specific myositis associated rash Features of CTD (raynauds, arthritis, etc.) Muscle enzymes 3-100x normal +ANA or myostis specific antibody

Bullous pemphigoid

Subepidermal bulla Dermal infiltrate w/ numerous eosinophils Autoimmune blistering disorder Occurs mostly in elderly 1/3 have oral involvement Flexural and intertriginous areas characteristically involved Bullae are large and tense Negative Nikolsky's sign (no extension w/ lateral pressure) Labs: biopsy, direct immunofluorescence (linear BM w/ IgG and C3) Therapy: topical steroids, antihistamines, prednisone and steroid sparing agents Path: Ab against hemidesmosome on basal keratinocytes (BP230 and BP180) Prognosis good with subsiding in months to years

Amyopathic DM

Subset of DM patients w/ cutaneous manifestations of DM for 6 mo or longer No clinical evidence of prox muscle weakness and no serum enzyme abn Synonymous w/ DM sine' myositis

Solar lentigo

Sun spots, age spots, liver spots Very common brown macule due to sun damage -esp on face, dorsum of hands -often multiple in older adults Not precancerous but can ID patients at risk Benign, hence no treatment necessary Cosmetic treatments: -bleaching creams -liquid nitrogen -chemical peels -lasers Histo: hyperpigmented, elongated rete ridges Solar elastosis

Folliculitis

Superficial bacterial infection of hair follicles Small erythematous sometimes pruritic pustules, may be sexually transmitted Mostly due to staph aureus, pseudomonas (hot tub folliculitis), or candidiasis Deep lesions need drainage Topical antibiotics: -bactroban Topical chlorhexidine or bleach baths -1/2 cup of clorox to tub of bathwater Oral abx may needed if deep or extensive

Erysipelas

Superficial cellulitis involving dermal lymphatics Beta hemolytic group A strep **local redness, heat, swelling, and a highly characteristic raised, induration w/ marked border** Prodrome of malaise, chills, high fever, headache, vomiting, arthralgias High PMN **affects legs and face, some may have systemic symptoms** Risks: operative wounds, fissures, abrasion/scratches, venous insufficiency, obesity, lymphedema, chronic leg ulcers Tx - systemic antibiotics to cover strep, ice bags and cold compress, leg involvement may require hospitilization and IV antbiotics Longer stay for elderly, compromised, longer duration of illness, leg ulcers

Pemphigus vulgaris

Suprabasilar split w/ characteristic "tomb stone" formation Acantholysis of keratinocytes Dermal infiltrate w/ scattered eosinophils Autoimmune disorder (desmoglien 3 in desmosomes of keratinocytes) Mucous involvement common Occurs in middle aged and older adults Vesicles are flaccid and rupture easily, leaving weeping and crusting erosions Pressure applied laterally results in extension Labs: biopsy, direct and indirect immunofluorescence: epidermal intercellular IgG and C3 (fishnet pattern) Therapy: prednisone and steroid sparing agents Associated w/ small cell lung cancer, non-hodgkin's lymphoma

Malignant melanoma: treatment

Surgical excision w/ appropriate margins Possible sentinel lymph node biopsy (recommended for depths over 1mm) Chemo for advanced disease TNM: ulceration automatically upstages T1: less than 1mm T2: 1-2mm T3: 2-4mm T4: over 4mm b: with ulceration Breslow depth is best prognostic indicator

SCC treatment

Surgical: -excision (standard for invasive SCC) +wide local or Mohs -Curette and desiccation (for SCCIS) Non surgical -radiation for poor surgical candidates -5-FU cream, imiquimod cream, photodynamic therapy (for SCCIS) Surveillance -Rx related complications -local and regional recurrence -monitor for development of new skin cancers

Joint examination: timing of symptoms after trauma

Swelling: Immediately following trauma -- think blood Latent -- indicates clear fluid in joint Pain Immediate -- fractures or internal derangement Progressive -- sprained connective tissue Intermittent, locking up, "giving way" -- internal derangement

Causes of polyarthritis - inflammatory

Symmetric -RA; JIA -Adult onset still's disease -SLE -Mixed connective tissue diseases -Polymyalgia rheumatica -Jaccoud's arthritis Asymmetric -ankylosing spondylitis -psoriatic arthritis -undifferentiated spondylartrhopathy -JIA (pauciarticular) -Palindromic rhematism

Causes of polyathritis - infectious

Symmetric -Viral Asymmetric -Bacterial -Bacterial endocarditis -Lyme disease (late phase)

Causes of polyarthritis - Misc

Symmetric -hypertrophic osetoarthropathy -amyloid arthropathy -myxedematous arthropathy -sarcoid arthritis (acute type) -cancer associated arthritis Asymmetric -cancer associated arthritis -pancreatic disease associated

Causes of polyartrhtis - degenerative/crystal induced

Symmetric -osteoarthritis -CPPD (pseudo rheumatoid) -Milwaukee shoulder -hemochromatosis arthropathy Asymmetric -gout -CPPD (pseudogout type)

JIA: polyarthritis, RF+

Symmetrical arthritis in five or more joints, resemble adult RA Large and small joints (knees, wrists, MCPs, PIPs, DIPs, ankles, MTPs, elbows, hips, C spine, TMJ, shoulders) Females > males Late childhood, adolescence Low grade fever, anemia, malaise, rheumatoid nodules Persistent, chronic, destructive, severe arthritis in over 50%

Limited systemic sclerosis

Symmetrical skin thickening limited to below the elbows and knees and involving face/trunk Progression is slow -- months to years after raynaud's onset Later and less severe involvement of internal organs Late development of PHTN Associated w/ anti-centromere antibodies Prognosis better w/ 70% survival at 10yrs

Polymyositis and dermatomyositis criteria

Symmetrical weakness of limb girdle muscles and anterior neck flexors Muscle biopsy evidence of necrosis of Type I and II fibers, phagocytosis, regeneration, variation in fiber type w/ inflammatory exudation Elevation in serum of skeletal muscle enzymes EMG triad of short, small polyphasia motor units, fibrillation and sharp waves; and bizarre, repetitive discharges Dermatologic features

Fibroblasts in proliferation

Synthesize matrix Activated by cytokines and growth factors produced by macrophages and platelets Feedback from matrix on migration, synthesis, proliferation

Osteoporosis: definition

Systemic skeletal disease characterised by low bone mass and deterioration of bone tissue, w/ increase in bone fragility and susceptibility to fractures

Bone mineral density

T score -- SD compared to young adult mean - used to define categories of osteoporosis Z score -- SD compared to age matched adults, may be a clue to presence of secondary osteoporosis (if outside of +/- 2) Treat T score of 1.5 or less

Lichenification

Thickening of the skin induced by scratching

Atrophy

Thinning of any part of the skin, depression of the surface occurs w/ time

Chronic tophaceous gout

Usually develops after 10 or more years of acute intermittent gout Intercritical periods no longer free of pain Involved joints are persistently uncomfortable and swollen Tophi develop over time (may be confused with rheumatoid nodules) Polyarticular involvement becomes more frequent

Patch testing

Tool for iding allergens in patients w/ allergic contact dermatitis Detects a delayed type IV hypersensitivity reaction Takes several days to develop TRUE test -- screening patch test series -meds -fragrances -preservatives -metals -rubber compounds -misc. chemicals Read at 48hrs for irritant rxns, read at 96 hours for true allergic rxns (MWF appts) Rxn grades: 1+ palpable erythema 2+ papules and vesicles 3+ bullae Positive result must be relevant to the eruption

Skin histology

Top: five transient layers of epidermis, basophilic staining portion of skin (don't worry about layer between granulosum and corneum) Stratum corneum (dried out keratin) from top to most basophilic line (stratum granulosum - NOT MELANIN, where keratinization occurs), thickness indicates thickness of skin Stratum spinosum -- daughter cells migrating upward from stratum germinativum Stratum germinativum -- undulating portion apposing the papillary dermis -- the mitotically active portion of epidermis Eosinophilic area --> dermis Upper portion is papillary dermis Lower portion (with thick collagen fibers coarsing through) is reticular layer; collagen important for wound healing Unilocular fat in hypodermis (not part of skin), if you see unilocular fat in a lesion, will require closure technique (suture, staples, etc) -- contains blood vessels and nerves

Dermatophytoses: treatments

Topical antifungals -econazole Oral antifungals -terbinafine -itraconazole Mentholated petrolatum Vinegar soaks

Bullous pemphigoid/pemphigus

Topical corticosteroids Oral immunomodulators/suppressants -prenisone -steroid sparing agent: mtx, azathioprine, cyclosporine, cyclophosphamide IV immunoglobulin Rituximab - anti CD20 Plasma/photopheresis

Acne: treatment

Topical medications -Antibacterials (clindamycin, erythromycin) -- addition of benzoyl peroxide reduces resistance -Retinoids (tretinoin, tazarotene, comedolytic) Systemic therapy: Antibiotics: -tetracycline -doxycycline -minocycline -TMP-SMX Hormonal (spironolactone, OCPs) Retinoids (isotretinoin for severe acne) Other: -laser -chemical peels -microdermabrasion -photodynamic therapy -intralesional corticosteroids

Transient synovitis

Transient effusion of hip Preceding URI in about half Age: 3-8 Male more than female Sx: -fevers rare -acute onset of hip pain/limp -resolves in 1-2 weeks to a month Treatment: NSAIDs

Wheal

Transient, edematous, plateau-like elevation of varying size; pink to red in color

Causes of hip pain: peds

Trauma Infections Inflammatory -transient synovitis -JIA -spondyloarthropathy Avascular necrosis Developmental -hip dysplasia -slipped femoral epiphysis Muscle strain Neoplasm Sickle cell pain crisis

Causes of knee pain: peds

Trauma Patellofemoral dysfunction Infectious Osgood schlatter Inflammatory (JIA, SA) Benign joint hypermobility syndrome Iliotibial band syndrome Prepatellar bursitis Plica syndrome Referred pain from hip pathology

Rosacea

Treatment Control of inflammation and papules/pustules Topical: -metronidazole -sodium sulfacetamide -azelaic acid Oral medications -doxycyline -minocycline -Isotretinoin Improvement in erythema -pulsed dye laser -green tint cosmetics Prevention of further damage -sunscreen -avoidance of trigger factors: red wine, spicy foods, etc...

Reactive arthritis

Triad: Arthritis, conjunctivitis (esp w/ shigella, salmonella, campylobacter), urethritis (also, salpingitis, vulvovaginitis, aseptic pyuria, prostatitis) Arthritis 1-3 weeks after venereal infections or gastroenteritis Mild constitutional symptoms, low grade fever but can appear toxic (high fever, weight loss, malaise) Typically in lower extremities, asymmetric, additive, can be after eye/GU symptoms resolve Enthesitis Sausage digits Low back pain/sacroiliitis, can radiate into buttock and thigh, worse w/ rest Pleurisy Extraarticular: -Keratoderma blenorrhagica - nodules, papules, vesicular rash -Circinate balanitis - shallow ulcers, asymptomatic -oral ulcers -GI inflammation resembline Crohn's -Aortitis -IgA nephropathy or amyloidosis Association w/ HIV Do not have to be HLA B27+

SLE: management

Try to treat by the most severe organ involvement -hydroxychloroquine -azathioprine -cyclophosphamide -mycophenolate mofetil -methotrexate -belimumab -IvIg -rituximab -autologous stem cell transplant For fatigue -stress reduction, sleep hygience and rest, steroids For serositis -salicylates, NSAIDS, low dose steroids Cutaneous lupus -sun protection, topical steroids, antimalarials, dapsone, thalidomide Arthritis, arthralgia, myalgia -NSAIDS, salicylates, steroids, plaquenil, methotrexate, belimumab,

Pressure ulcers: management

Turning around the clock Bed surface doesn't matter Avoid shearing forces GRAVITY IS THE ENEMY Stage I - transparent film Stage II - petroleum based non adherent (vasoline gauze), hydrocolloid Stage III/IV - alginate, gauze packing, hydrogel Relieve pressure Remove necrotic tissue Protect clean wound base Granulation should appear w/in time frame for wound healing (7-10 days) Be alert for secondary infection Never send a wound swab culture

Osteoporosis types

Type I senile -- bone resorption greater than bone formation, bone formation levels low Type 2 post menopausal, bone resorption more than formation, formation levels high Treatment can be aimed at slowing resorption or increasing formation Resorption more common target

Straight cane use

Typical cane, w/ J handle Like the whiskers on the cat, useful in adding another point for proprioception Ex: vision problems (glaucoma, cataracts), mild peripheral neuropathy (occasional missteps, but not falling), mild dizziness, mild balance problems

Diabetic foot ulcer

Typically on ball of foot or other weight bearing pressure point Hard keratinized margin w/ small deep open center Small vessel disease, neuropathy Debridement superficial only for comfort Probe for bone Osteomyelitis more likely, empiric antibiotic warranted Wound cultures unhelpful

HSV: diagnosis

Tzanck smear will demonstrate multinucleated giant cells Viral culture Direct fluorescent Ab

Vitamin D and osteoporosis

UVB converts cholesterol to D3, hydroxylated in liver and kidney to active form Acts on intestine and bone to increase calcium and phosphorus absorption from gut and to mobilize Ca from bone (to maintain serum Ca and phosphorus)

Angiosarcoma

Uncommon, neoplasm of endothelial cells Starts as bruise like patch, may progress to violaceous elevated ulcerated nodule Often on head and neck, may arise in setting of chronic lymphedema Prognosis is poor

Alopecia treatment

Under age 10: -topical steroids -minoxidil solution or foam -short contact anthralin Over 10 w/ limited scalp involvement: -intralesional corticosteroids -topical steroids -minoxidil -short contact anthralin -scap prothesis (wig) Over 10 w/ diffuse involvement -topical immunotherapy -topical steroids -minoxidil -short contact antralin

Rosacea: pathogenesis

Unknown but most patients have an abnormal cutaneous vasomotor response to thermal and other stimuli Chronic sun damage also contributes (fair skinned patients most affected)

Psoriatic arthritis

Unknown cause, genetic/immunologic/environmental Male = female, onset in 20-40 Not all patients w/ psoriasis and arthrtitis have psoriatic arhritis, no relation between severity of skin and joint inflammation, may proceed skin involvement Clinical: -Distal arthritis -asymmetric oligoarthritis -symmetric polyarthritis -spondylarthropathy -arthritis mutilans Patterns not permanent, most will change from initial pattern Less tender than RA Spondyloarthropathy in 20-40% of pts but rarely at onset, usually in older men (more severe in men) Unilateral sacroiliitis Sausage digits Tenosynovitis Conjunctivitis, iritis Aortic regurgitation in late disease (rarely) Nails - pits Onycholysis (separation of nail from bed) Oil drop sign ( Nail lesions significantly associated w/ PA

Pressure ulcers: pathophys

Unrelieved axial pressure of 4-6 times systolic blood pressure causes necrosis in as little as one hour, pressure similar to systolic can take 12 hrs Shear pressure causes ulcers at lower axial pressures (raising head of bed) Moisture predisposes to breakdown Friction contributes to ulceration Extrinsic pressure >>>> capillary pressure

GI involvement: myopathy

Upper dysphagia - difficulty in initiating swallow or nasal regurgitation Cricopharyngeal muscle dysfunction more common in inclusion body myositis -blocking sensation or retrosternal sticking GI mucosal ulceration and hemorrhage rare -more common in JDM, can be life threatening

Eccrine sweat glands

Where dermis meets hypodermis Glands round, bigger, stain lighter Ducts have stratified cuboidal epithelium, stain intensely basophilic, move through all layers of skin to drain at level of epidermis

Pediatric lupus

Widespread inflammation of blood vessels and connective tissues Nearly all patients ANA+ Adolescent females Constitutional symptoms: malaise, fatigue, fever, anorexia, weight loss Renal disease (may be presenting issue) CNS disease (seizure may be presentation) Susceptibility is multifactorial: genetics, complement deficiency, environmental (smoke, UV light, diet, meds) ("I'M DAMN SHARP") Poor prognosis, higher mortality in juvenile arthritis

ACS2: TSS

Woman of childbearing age, acute onset (6 hrs) of fever and rash Diffuse, macular rash covering abdomen and thorax, high fever and HR, low BP, dizziness, acutely ill Nafcillin

Indications for BMD testing

Women 65+ and men 70+ Age 50-70 w/ risk factors Fracture after 50 Disease or med associated w/ low bone mass or loss Taking or being considered for treatment Women discontinuing estrogen not recommended for children or healthy young adults

Osteoporosis treatment

Women and men age 50+ w/ : -hip or vertebral fracture -osteoporosis -osteopenia and prior fractures or secondary causes w/ high risk of fractures -WHO fracture risk of 3% or more hip or 20% or more major osteoporosis related fracture risk Bisphosphonates Calcitonin Estrogen replacement SERMs (raloxifene) Denosumab PTH

Psoriatic arthritis - imaging

XR changes early in course suggest aggressive disease or present for longer duration Erosive changes and new bone at distal joints, "pencil in cup" IMAGE C Unilateral sacroiliitis C spine involvement Bony spurs/periosteal reaction Marginal syndesmophytes (bony growth resulting from enthesitis)

ACS2: Kawasaki

Young child, fever, rash, conjunctivitis, sore throat, cracked red lips Bilateral LAD, maculopapular rash on trunk w/ erythema of palms and soles Coronary artery aneurysm as complication

Acrodermatitis enteropathica

Zinc deficiency Scaly erythematous eczematous to macerated/erosive plaques on perioricial, acral, scalp distribution Treatment: zinc for life

Drug induced hypersensitivity syndrome (DIHS)

aka DRESS (drug rxn w/ eosionphilia and systemic symptoms) Patho: certain HLA type, altered drug metabolism, increased IL-5, reactivation of HHV 6&7, EBV and CMV Common drugs: antivonvulsants, antibiotics (sulfas, minocycline/vanc, penicillin, metronidazole, dapsone), allopurinol, anti HIVs, gold salt/NSAIDs/isoniazid Sx: 2-6 after drug admin, morbilliform (measle like) eruption, fever, facial edema, LAD, arthralgias, systemic involvement (eosinophilia, LFTs, CNS, kidney, lungs, heart, thyroiditis), rash and malaise may perisist up to a year Consult derm; Withdraw suspect drugs and stop non essential meds, consider systemic steroids, topical steroids antihistamines

Heberden's nodes

hard, bony swellings of DIPs May be associated with overlying synovial cyts

PAN

multisystem necrotizing vasculitis of small and medium vessels No pulm involvement Lesions are segmental, occur at bifurcations/branching of arteries Causes: -intimal proliferation -wall degeneration -fibrinoid necrosis --> lumen compromise --> ischemia Aneurysmal dilatations Hep B association Sx: Constitutional -- fever, weight loss, malaise GI -- abd pain, n/v, GI bleeding MSK: arthralgia, arthritis, myalgia Renal: HTN, hematuria, insufficiency Skin -- purpuric rash, nodules, livedo reticularis, cutaneous infarcts, raynaud's GU -- testicular or ovarian pain Neuro -- mononeuritis multiplex, CVA, seizures, altered status

Scar

new connective tissue that replaces lost dermis or deeper skin as a result of injury or disease