Bolognia KODAS - All

acanthamebiasis in HIV - dissemination to the skin and CNS can occur in profoundly immunocompromised patients; the usual cause is Acanthamoeba castellani, a microorganism that is part of the normal oral flora. - Necrotic nodules and painful ulcerations on the trunk and extremities develop in these patients. - Careful inspection on tissue sections will reveal amebic cysts, trophozoites and histiocyte-like cells with erythrophagocytosis. - responds to metronidazole

acanthamebiasis in HIV

seborrheic dermatitis in HIV (cd4 > 500) - most common skin disorder to affect HIV- infected individuals (up to 85%) and is seen in all stages of the disease - clinical presentation is the same but may have exaggerated or sudden/acute onset, exuberant facial plaques

acute onset

actinic folliculitis - development of follicular pustules on the upper trunk and arms 24-30 hours after first sun exposure of the summer, which spare the face and are not pruritic - sun avoidance; sunscreens will not prevent lesion formation - topical corticosteroids or low dose Isotretinoin

after sun exposure

- MRSA most commonly manifests as furunculosis, which may evolve into large abscesses, cellulitis or necrotic plaques - methicillin resistance is the production of an altered penicillin-binding protein (PBP), called PBP2a, with decreased affinity for β-lactam antibiotics (which interfere with bacterial cell wall synthesis by binding to PBPs). - PBP2a is the protein product of the mecA gene, which is located within a specific mobile genetic element called the staphylococcal cassette chromosome mec (SCCmec). - Acquisition of different SCCmec elements by S. aureus led to the emergence of MRSA - doxycycline, minocycline, bactrim, clindmycin, linezolid, vancomycin

+MRSA

bite like reactions in CLL - Papulovesicular and nodular lesions resembling bites have been described in patients with chronic lymphocytic leukemia and (less frequently) other hematologic malignancies as fell as chronic EBV and EBV carrying NK lymphocytosis - Although the lesions frequently appear months after the diagnosis of leukemia and are unrelated to its course or therapy, they can also herald development or recurrence of leukemia or lymphoma. - The clinical appearance, distribution and histologic features (including the presence of degranulating eosinophils) of the lesions suggest that they represent exaggerated bite reactions; although patients often do not recall having been bitten - screen for hematologic malignancy, EBV, and NK cell lymphocytosis in patients with unusual arthropod bite reactions

60 yr old with numerous "bites"

eosinophilic pustular folliculitis in infancy - self limiting condition of infancy (< 1 yr) with multiple, pruritic, follicular pustules and vesicopustules on an erythematous base on the scalp and brow with secondary crusting - may cycle before burning out after a few years - characterized by infundibular eosinophilic pustules - topical steroids for symptomatic itching

< 1 yr old

CMV in HIV (cd4 < 50) - Common clinical manifestations include retinitis, esophagitis and colitis - cutaneous disease is relatively uncommon; presentations include ulcers (especially in the anogenital area), verrucous or hyperpigmented plaques, purpuric papules, vesicles and morbilliform eruptions - biopsy shows owls eye intranuclear inclusions of endothelial cells

CMV in HIV

Candidiasis in HIV - susceptible to oral, perleche, disseminated - Conventional therapy (e.g. oral fluconazole) is indicated when the patient is symptomatic42; however, persons with CD4+ counts <50 cells/ mm3 should be advised that excessive antifungal use can promote resistance.

HIV

Dermatophytes in HIV - Cutaneous involvement can be atypical in appear- ance, and lesions may be more widespread and resistant to therapy. - Spread of interdigital tinea pedis onto the dorsal foot, Majocchi's granu- loma (a deep folliculitis with rupture of the follicle wall) and proximal white onychomycosis have all been associated with HIV infection - The goals are to eradicate the infection as well as to prevent recurrence. - The latter includes local measures such as absorbent powders

HIV

Eosinophilic folliculitis in HIV (cd4 < 200) - exaggerated reaction to Malassezia yeast or other organisms normally present within the follicular infundibula and is a reflection of immune dysregulation leading to a Th2-predominant immune response. - Excoriated follicular papules and rare intact pustules are found primarily on the face and upper trunk - Cultures are negative and peripheral eosinophilia may be present - ART, topical steroids, UVB

HIV

NMSC in HIV - HIV-infected individuals have a three- to vefold higher risk of developing these non-melanoma skin cancers, which tend to appear at a younger age and are more often multifocal and located on the trunk and extremities - Cutaneous SCCs in HIV-infected individuals have a high risk of recurrence and metastasis.

HIV

PRP in HIV - Pityriasis rubra pilaris (PRP) can develop in conjunction with HIV infection (referred to as type VI PRP) - PRP is typically generalized and may occur together with follicular spines, acne conglobata and hidradenitis suppurativa (HIV-associated follicu- lar syndrome).

HIV

Scabies in HIV - Severe infestation may develop as a consequence of diminished cell-mediated immunity. - Cutaneous lesions vary from the "classic" crusted papules to pruritic dermatitis to keratotic and crusted plaques; burrows are often less apparent - Secondary infection with bacteremia and fatal septicemia has been reported in HIV-infected patients with scabies - keep high index of suspicion for scabies in an itchy HIV patient - topical permethrin, oral ivermectin, 6% sal acid (for crusted) - Mites under the nails, failure to treat contacts and failure to dry laundered bed linens and clothing at high temperatures are common causes of reinfestation

HIV

psoriasis in HIV - psoriasis tends to be more severe in HIV: It may develop at any stage of HIV infection; "eruptive" psoriasis can serve as a clue to an underlying HIV infection, and psoriasis tends to worsen with declining immune status. - topicals, phototherapy and systemic retinoids are first line treatments - Immunosuppressive agents such as methotrexate, cyclosporine and TNF-α inhibitors should be used with caution in HIV-infected patients.

HIV

HIV pruritus - pruritus is common AIDS patients and also HIV infected people are more likely to get pruritic papular eruption, eosinophilic folliculitis, severe seborrheic dermatitis, psoriasis, scabies, drug eruptions, xerosis, and acquired ichthyosis - may have elevated IgE and eosinophils - HAART may or may not receive pruritus - topical steroids, anti-histamines, NBUVB, thalidomide

HIV with pruritus

Vaccinia - Papule at the vaccination site (usually deltoid or thigh) develops into vesiculopustules, then crust - Occasionally, satellite lesions

at site of small pox vaccine

EBS with muscular dystrophy - AR form of EBS due to mutations in the gene encoding plectin, which is expressed in skeletal muscle as well as in the hemidesmosomes of basilar keratinocytes - prevention of infection through bathing or soaking with 0.005% sodium hypochlorite (0.5 cup household bleach in a full standard bathtub) or 0.25% acetic acid (1 part white vinegar [5% acetic acid] to 20 parts water) may help to reduce bacterial colonization. - non-adherent soft silicone dressing (Mepilex, Mepilex border); Silver-impregnated dressings may be helpful for heavily colonized or infected wound (Mepliex AG)

blistering on hand and feet with progressive, insidious muscle weakness

burning mouth syndrome - burning mucosal pain without clinically detectable oral lesions. - the pain is typically bilateral, often involving the anterior two-thirds of the tongue, palate and lower lip; and rarely involves the buccal mucosa and floor of mouth - associated with anxiety and depression - evaluate for Ill- fitting dentures, oral candidiasis, xerostomia, malignant - amitriptyline (10-75mg), doxepin, gabapentin

burning

burning scalp/scalp dysesthesia - diffuse burning pain, pruritus, numbness or tingling of the scalp without objective findings - associated with anxiety and depression - eval for seb derm, LPP, DLE, folliculitis - amitriptyline (10-75mg), doxepin, gabapentin, topical capsaicin

burning

genital dysesthesia - pain/burning without cutaneous abnormalities - exclude infection, KOH, ensure not dermatographic - oral/topical gabapentin, topical lidocaine, amitriptyline (10mg qHS and titrate up to 100mg qHS if needed)

burning, no rash

Centipedes - Centipedes are fierce hunters who inject a neurotoxic venom through venom ducts in their jaws - they have long, worm-like bodies that are flattened dorsoventrally with 17 or more segments; up to 20 cm in length - Each segment, except the last one, has one pair of legs - Pain, edema and erythema at bite sites and occasionally with profuse bleeding - Ice packs, analgesics and antihistamines - observe for 4 hours to monitor of systemic symptoms

centipede

Milkers nodule - pox virus are double-stranded DNA viruses that are characterized by cytoplasmic replication and cause smallpox, vaccinia, cowpox, monkeypox, tanapox, orf, milker's nodules and molluscum contagiosum (MC) - Papule develops at site of contact with lesions on infected animals (e g muzzles of calves, teats of cows) • Often single lesions; favor hands and forearms • Cutaneous lesions virtually identical to orf

contact with cattle

Orf - pox virus are double-stranded DNA viruses that are characterized by cytoplasmic replication and cause smallpox, vaccinia, cowpox, monkeypox, tanapox, orf, milker's nodules and molluscum contagiosum (MC) - Papules (1 to few) on hands at sites of contact with infected animals (sheep; goats; where lesions favor perioral area and udders of ewes), carcasses - Progression through several stages: maculopapular -> targetoid -> weeping nodule -> regenerative dry stage with black dots -> papillomatosis -> regression with a dry crust - May be accompanied by lymphangitis, lymphadenopathy, malaise, fever - PCR or biopsy which shows epidermal necrosis, vacuolated keratinocytes, eosinophilic inclusion bodies (intracytoplasmic > intranuclear) - self resolves

contact with goats and sheep

diabetes pruritus - generalized pruritus is possible, but not more common than in the general population - anogenital pruritus can be associated with poor glycemic control - neuropathy can cause pruritus

diabetes pruritus

dysesthetic anogenital pain - chronic or recurrent anogenital pain but no detectable abnormalities on physical examination - r/o hemorrhoids, fissures, trauma, infection (e.g. chlamydia, herpes simplex, candidiasis) and (in men) testicular torsion, and malignancy

dysesthetic anogenital pain

Wiskott Aldrich - X-linked recessive immunodeficiency disorder due to mutation in WASP gene which controls the assembly of actin filaments, which are required for microvesicle and pro-platelet formation as well as T-cell activation and polarization toward antigen-presenting cells - characterized by the classic triad of atopic-like dermatitis, a bleeding tendency due to microthrombocytopenia, and recurrent sinopulmonary infections - first clinical signs of WAS are usually petechiae and ecchymoses of the skin and oral mucosa due to platelet dysfunction - Onset of bacterial infections is early in life, with later development of viral and Pneumocystis spp. infections - Hepatosplenomegaly, lymphadenopathy and autoimmune diseases often develop - Lymphomas occur in approximately one-quarter of survivors - cause is death is infection > bleeding > lymphoma - requires stem cell transplant; topical steroids, antibiotics

eczema with petechiae

strep toxic shock - rapidly progressive, life-threatening illness caused by infection with toxin-producing group A streptococci - usually affects healthy individuals between 20 - 50 years of age with invasive strep infections; bacteria hematogenously disseminates and produces toxin which binds directly to MHC II and TCR and causes massive release of cytokines and clonal T-cell expansion - flu-like symptoms with variable blistering and macular erythema progressing to shock and multiorgan failure usually develop 48-72 hours; +/- desquamation hands - isolate strep from sterile skin, often pos blood cultures - ICU + surgical intervention of soft tissue infections - penicillin antibiotics, +/-clindamycin (suppress toxin), +/- IVIg (neutralize toxin); mortality 30-60%

fever and hypotension

Penicilliosis - a Hyalohyphomycosis opportunistic infection caused by Penicillium - the molds is found in the soil and throughout the environment in SE Asia and is spread by the bamboo rat - can cause disseminated infections in immunocompromised hosts (neutropenia, HIV) - umbilicated papules that resemble molluscum contagiosum, necrotic nodules and acneiform lesions on the face (particularly the forehead), arms and trunk. - biopsy to search for small organisms found within macrphages and giant cells

from SE asia

Tarantulas - Typically non-aggressive large hairy spiders, up to 15 cm in diameter, and found in the southwestern US - Many species of tarantulas possess urticating hairs in a characteristic patch on the dorsal abdomen. These hairs are used in a defensive fashion to drive predators from the spider's burrow. Vibrations of the hind legs are used to flick hairs at the perceived attacker. - Itching at the site of urticating hair penetration may persist for several weeks after exposure. - Hairs that penetrate the cornea can result in ophthalmia nodosa, a chronic granulomatous reaction that can lead to loss of vision. - topical steroids and anti-histamines

got a new pet tarantula

Chédiak-Higashi syndrome (CHS) - AR mutation in the lysosomal trafficking regulator gene (LYST) of vesicle trafficking that results in giant organelles, including melanosomes, leukocyte granules and platelet-dense granules - Silvery hair (due to small, regular clumps of melanin), mild diffuse pigmentary dilution (often with an admixture of hyper- and hypopigmentation in sun-exposed sites), and a variable degree of photophobia and nystagmus is present in infancy - Recurrent pyogenic infections and a mild bleeding diathesis - An "accelerated phase" with pancytopenia and organomegaly due to uncontrolled T-cell and macrophage activation (against large vesicles?) leads to death by 10 yrs, unless hematopoietic stem cell transplantation - Progressive neurologic deterioration in survivors

hair has small regular clumps of melanin

African tick bite fever - One of the most common rickettsial diseases in travelers is African tick bite fever, which is seen in those who have been on safari or bush walking in southern Africa due to Rickettsia africea - one or multiple escars at site of inoculation with fever, myalgias, and possible morbilliform rash

has been walking around in Africa

mixed connective tissue disease - combination of features from rheumatoid arthritis, systemic sclerosis, systemic LE and polymyositis - high titer anti-U1RNP antibodies, Raynaud's phenomenon with digital ulcers , swollen hands or sclerodactyly, myositis, esophageal dysmotility and arthritis - pulmonary hypertension is most serious complication - topical steroids and plaquenil for skin - gloves and sildenafil for Raynauds - immunosuppressives for systemic involvement

high titer anti-U1RNP antibodies

rat bite fever - Streptobacillus moniliformis infections resulting in an acute illness characterized by fever, arthritis and a rash - caused by bite from infected rat or living in close contact with rodents, or ingestion of contaminated foods - seen in urban areas with poor sanitation where there is a large population of rats - ulcer at site of bite with lymphadenopathy, paroxysmal fevers, and migratory polyarthritis - then develop develop characteristic acral eruption involving the palms and soles of morbilliform macules and papules, petechiae, vesicles, pustules and crusts may be seen - penicillin for 1 week

homeless man with fever and migrating arthritis

Tularemia - bacterial infection caused by Francisella tularensis, a Gram-negative, non-motile coccobacillus - cutaneous inoculation via tick or deer fly bites or contact with infected rabbits and their carcasses - ulceroglandular tularemia is the most common form and is characterized by both lymphadenopathy and an erythematous, indurated, punched-out ulcer at the site of inoculation that may last for several weeks; can progress to potentially fatal infection - diagnosis made via fluorescent antibody testing - 10-day course of streptomycin (the classic first-line agent), gentamicin or a quinolone - A Jarisch- Herxheimer-like reaction may occur following the initiation of therapy

hunter

eosinophilic folliculitis - recurrent crops of very itchy red follicular papulopustules on the "acne" areas - can be seen in the Japanese (ofujis's disease w/ peripheral eosinophilia) or in the immunosuppressed (cd4 < 400, leukemia/lymphoma) - characterized by infundibular eosinophilic pustules - topical antipruritics, topical steroids, NBUVB, raise cd4 count

in a patient with AIDS

Malakoplakia - a chronic granulomatous infection with distinctive macrophages that are unable to phagocytose and kill bacteria effectively (E. coli, although Pseudomonas, Proteus, Klebsiella, Staph and Mycobacterium). - occurs in immunocompromised hosts, particularly solid organ transplant recipients, in the GU tract - cutaneously, can get ulcerations, abscesses with multiple draining sinuses, yellow-to-pink soft papules, and erythematous indurated nodules in the anogenital regions - von Hansemann cells are large macrophages that contain Michaelis-Gutmann bodies - excision, long term antibiotics

in a solid organ tranplant patient

PPK - Palmoplantar keratodermas can be inherited or acquired - The three major patterns of involvement are diffuse (entire palmar surface), focal (nummular with oval lesions and striate with linear lesions) and punctate (small keratotic papules) - Additional distinguishing features include an erythematous border, associated hyperhidrosis and transmigration to areas beyond the palmoplantar skin - it is important to determine whether other features are present, e.g. impaired hearing, cardiomyopathy (in patients with woolly hair), starfish keratoses, pseudoainhum, knuckle pads, atrophy, oral lesions, nail changes, blisters - Histologic examination can determine whether or not epidermolytic hyperkeratosis is present

in general

Rickettsialpox - infection with Rickettsia akari from the Liponyssoides sanguineus (mouse mite in cities in Northeast USA from parasitized mice living in houses) - Rickettsiae resides in an arthropod host (tick, flea, louse or mite) and is transmitted in its saliva or feces during feeding - in humans, attach to vascular endothelial cells leading to vascular permeability, edema, hypovolemia/tension - A papulovesicle arises within 48 hours at the site of bite which eventuates into an eschar; then 1-2 weeks later get fever, HA, myalgia followed in 2-3 days by diffuse erythematous macules/papules/vesicles or hemorrhagic crust (20-40 lesions) - circulating rickettsiae are generally too few early in the illness to be detected by PCR-based assays, but rickettsiae can be seen by ICH on biopsy specimen of an eschar or a maculopapular lesion, especially petechial. - lymphohistiocytic vasculitis is seen on h&e of petechiae - doxycycline; chloramphenicol if pregnant

lives in Boston

Mediterranean spotted fever - infection with Rickettsia conorii from the Rhipicephalus (brown dog tick in Southern Europe and Asia) - Rickettsiae resides in an arthropod host (tick, flea, louse or mite) and is transmitted in its saliva or feces during feeding - in humans, attach to vascular endothelial cells leading to vascular permeability, edema, hypovolemia/tension - eschar at site of bite (necrosis surrounded by erythema called tache noir) with fever, HA, myalgia, then get erythematous to petechial papules over lower limbs - circulating rickettsiae are generally too few early in the illness to be detected by PCR-based assays, but rickettsiae can be seen by ICH on biopsy specimen of an eschar or a maculopapular lesion, especially petechial. - lymphohistiocytic vasculitis is seen on h&e of petechiae - doxycycline; chloramphenicol if pregnant

lives in Croatia

Epidemic louse borne Typhus - infection with Rickettsia prowazekii from the feces of human body louse (Pediculus humanus var corporis) in S America and Eurasia - Rickettsiae resides in an arthropod host (tick, flea, louse or mite) and is transmitted in its saliva or feces during feeding; feces get rubbed into skin when scratch - in humans, attach to vascular endothelial cells leading to vascular permeability, edema, hypovolemia/tension - fever, myalgia, HA then 5 days later get erythematous to petechial macules on central chest that spread outward, sparing the face, palms, and soles - circulating rickettsiae are generally too few early in the illness to be detected by PCR-based assays, but rickettsiae can be seen by ICH on biopsy specimen of an eschar or a maculopapular lesion, especially petechial. - lymphohistiocytic vasculitis is seen on h&e of petechiae - doxycycline; chloramphenicol if pregnant

lives in S. America

Endemic typhus - infection with Rickettsia typhi from the feces of rat flea (Xenopsylla cheopis) world wide, including USA - Rickettsiae resides in an arthropod host (tick, flea, louse or mite) and is transmitted in its saliva or feces during feeding; feces get rubbed into skin when scratch - in humans, attach to vascular endothelial cells leading to vascular permeability, edema, hypovolemia/tension - fever, myalgia, HA then 5 days later get erythematous to petechial macules on central chest that spread outward, sparing the face, palms, and soles - circulating rickettsiae are generally too few early in the illness to be detected by PCR-based assays, but rickettsiae can be seen by ICH on biopsy specimen of an eschar or a maculopapular lesion, especially petechial. - lymphohistiocytic vasculitis is seen on h&e of petechiae - doxycycline; chloramphenicol if pregnant

lives in US

Mycobacterium in hiv - M tuberculosis, Mycobacterium avium complex, M. kansasii, M. haemophilum and M. fortuitum may also produce skin lesions. - Erythematous papules and nodules, ulcers, verrucous plaques and deep nodules can all be observed. - Pruritus is generally absent and patients may be febrile. - ART and appropriate prophylaxis ( bactrim, pentamidine if cd4 < 200)

mycobacteria in HIV

Keratoderma Climactericum, acquired PPK - Hyperkeratosis begins over pressure points on the heels in women over the age 45 with no preceding history of skin disorders, including eczema or psoriasis. - As the hyperkeratosis extends, fissures form and make walking painful. - Obesity and cold dry climates aggravate the condition, as can wearing backless shoes, e.g. sandals. - If present, involvement of the hands is much milder and more discrete. - oral retinoids, Topical estradiol 0.05% ointment, 25-40% urea

new onset in older woman

cutaneous anthrax - Bacillus anthraces (gram positive rod) causes pulmonary (inhalation), gastrointestinal (ingestion) and cutaneous disease (inoculation) [cutaneous disease accounts for 95% of cases] - cutaneous lesions are toxin mediated: Edema toxin impairs neutrophil function and affects water homeostasis, leading to edema; lethal toxin causes release of TNF-α and IL-1β - Cutaneous anthrax has traditionally been considered an occupation-related condition; those who handle infected sheep, cows, horses, goats or their carcasses - A purpuric macule or papule develops in an exposed area (may resemble an insect bite and can be pruritic), then a vesicle which ulcerates and small vesicles may form around the ulcer - The lesion becomes hemorrhagic and depressed, and a painless, black necrotic eschar forms which then dries and sloughs off - ciprofloxacin 500 mg orally twice daily for 60 days because of possible concomitant inhalational exposure

no pain

Cowpox - pox virus are double-stranded DNA viruses that are characterized by cytoplasmic replication and cause smallpox, vaccinia, cowpox, monkeypox, tanapox, orf, milker's nodules and molluscum contagiosum (MC) - Cowpox presents with painful papule at site of contact with lesions on infected animals (usually cats, no longer associated with cows) - Erythematous, edematous papule rapidly becomes vesicular and progresses to ulcer with crust and eschar - Lymphadenopathy, fever and flu-like symptoms - heals spontaneously with depressed scar

owns a cat

Brucellosis - a chronic granulomatous disease caused by Brucella, a genus of Gram-negative coccobacilli, in the Middle East - Transmission occurs through the consumption of contaminated unpasteurized milk products (commonly raw goat's milk and cheese), direct contact with infected animal parts, or inhalation of aerosolized particles - nonspecific signs and symptoms, malodorous perspiration, and in <10%, disseminated eruption of violaceous papulonodules, EN, vasculitis - Doxycycline

patient handles goats

gram negative folliculitis - pustules in the facial T-zone and in a perinatal distribution in a patient on long term antibiotics for acne - topical gentamicin or benzoyl peroxide, oral quinolones (e g cipro noxacin), and if severe or recalcitrant then isotretinoin, 0 5-1 mg/kg/day for up to 16 weeks

patient on long term antibiotics for acne

Papillion Lefevre syndrome - Loss-of-function AR mutations in the cathepsin C gene (CTSC) which is a lysosomal protease involved in immune function - diffuse, transgredient PPK in association with destructive periodontitis (beginning in childhood) and premature loss of teeth - skin infections and scaly psoriasiform lesions - calcification of fall/tentorium (dura)

periodontal disease

Hodgkin disease pruritus - nocturnal generalized pruritus in association with chills, sweating and fever - Reed Sternberg cells produce IL-5 to promote eosinophils, histamine release from basophils - treat lymphoma, topical corticosteroids, and oral mirtazapine (7.5-30 mg/ day)

persistent itch

Chikungunya virus - togavirus in Asian and Africa spread by Aedes mosquito - like dengue fever, patients develop fever, headache, myalgias, nausea, vomiting and a rash, but there is no associated hemorrhagic component or shock syndrome. - cutaneous manifestations include morbilliform eruptions; acral or facial (sometimes localized to the nose) erythema and edema; genital, intertriginous and oral ulcers; and prominent postinflmmatory hyperpigmentation on the face and extremities - characterized by low back pain and debilitating arthraligias

rash and back pain

WHIM syndrome - a rare, autosomal dominant primary immunodeficiency disorder characterized by HPV-induced warts (cutaneous and genital), hypogammaglobulinemia, recurrent bacterial infections and neutropenia due to myelokathexis (retention of mature neutrophils in the bone marrow) - It is caused by mutations in the gene encoding the chemokine CXC receptor 4 (CXCR4), which result in impaired leukocyte trafficking and homing

recurrent bacterial infections and neutropenia

Recurrent folliculitis - application of mupirocin 2% ointment twice daily to the nares for 5-10 days can be used to eradicate nasal carriage of S. aureus - methods to decolonize the skin (e.g. axillae, perineum/groin, submammary area) include topical mupirocin, washes containing chlorhexidine or triclosan, and dilute sodium hypochlorite baths (e.g. 0.5 cup house- hold bleach [6% sodium hypochlorite] in a full standard bathtub). - Elimination of bacterial contamination of potential fomites such as keyboards, toys and sports equipment (e.g. shoulder pads, wrestling mats) should also be considered (e.g. using ethanol- or sodium hypochlorite-based disinfectants).

recurrent disease

Hypohidrotic ectodermal dysplasia with immune deficiency - X-linked recessive pattern due to mutations in the NEMO (nuclear factor-κB essential modulator) - features of HED: sparse or absent hair (scalp, eyebrows/lashes, but retain sexual hairs); missing or peg- shaped teeth; and decreased ability to sweat - recurrent pyogenic bacterial (especially of the skin and respiratory tract) and oppor- tunistic infections, and it is characterized by a poor antibody response to polysaccharide antigens, dysgammaglobulinemia (typically with ele- vated IgM and IgA but decreased IgG levels), and defective natural killer cell activity.

recurrent skin infections

Scorpions - Scorpions are typically found under tabletops, in woodpiles and in shoes. - They sting when disturbed and accidentally trapped by a hand or foot. - they have large anterior claws and the long tail is curved upward to sting when threatened - pain out of proportion to erythema at sting site - ice is sufficient for most envenomations

scorpion

Menkes Kinkly hair disease - X-linked recessive disorder of copper metabolism, due to mutation in mutations in ATP7A which affects copper ATPase - affected males having pili torts (and trichorexis nodosa), severe psychomotor retardation, growth failure, seizures and other neurologic abnormalities - hair is sparse in density, depigmented, lusterless, and looks and feels like steel wool - serum copper will be low - most die in childhood

steel wool hair

Strongyloides in HIV - an intestinal helminth - HIV-associated cutaneous strongyloidiasis may be caused by larval penetration of the skin and super cial veins giving rise to the migrating serpiginous urticarial eruption known as larva currens. - Dissemination (i.e. hyperinfection) may occur in immunosuppressed individuals, and, when the skin is affected, lesions can mimic a number of other conditions such as urticaria and livedo reticularis; on the lower trunk, they may resemble purpuric "thumbprints"

strongyloides in HIV

thyroid pruritus - generalized pruritus may be seen in hyperthyroid due to direct effect of thyroid hormone on skin - may be seen in hypothyroid in form of xerosis and asteatotic eczema

thyroid pruritus

photo allergic reaction - a delayed-type hypersensitivity reaction with UVR induced photo allergen formation presenting as a pruritic eczematous eruption (like allergic contact dermatitis) - topical sunscreen most common (benzophenone/oxybenzone), also NSAIDs, promethazine, and fragrances - confirm with photo patch testing - consider airborne contact but will not typically spare the upper eyelids, the nasolabial folds, the retroauricular areas, the submental region - avoid the agent or take evening dose of medications such that their peak systemic levels occur during the night, photo protection, topical/systemic steroids

very itchy

WILD syndrome - a recently characterized disorder that features warts, cellular immunodeficiency, primary lymphedema and multifocal anogenital dysplasia. - The patient reported had extensive warts, including condylomata acuminata and lesions that clinically (but not histo- logically) resembled those in EV. - A variety of α HPV types were detected in the cutaneous and genital lesions

warts and lymphedema

Widow spider - Black widows are large, shiny black spiders with a round abdomen; a red hourglass shape is seen on the ventral abdomen of females (only females are capable of envenomation) - Widow spiders are typically found in woodpiles, in shoes and under outhouse seats; human envenomation usually occurs when the spider's environment is disturbed, and the spider is inadvertently trapped or pressed against the skin. - the venom contains latrotoxins that act by depolarizing neurons - Acute pain and edema occurs at the bite site with systemic symptoms possible including muscle spasms, mimic acute abdomen and rhabdomyolysis/tetany - rest, ice, elevation for bite site - Benzodiazepines and intravenous calcium gluconate for tetany; antivenom

with HA, muscle spasms, abdominal pain

HPV in HIV (cd4 < 500 for dysplasia) - Lesions may be extensive, with multiple ver- rucae on the face, limbs and genitalia that coalesce into large plaques; bowenoid papulosis can also develop - HIV-infected individuals may develop squamous cell carcinomas (SCCs) in associa- tion with AEDV and other cutaneous (e.g. digital related to high-risk HPV types) as well as anogenital warts - ART does not clear HPV - monitor anogenital lesions with serial proctoscopy and biopsy - destruction and/or imiquimod - quadrivalent HPV vaccine (GardasilTM) is safe and effective in HIV population

with HIV

Hartnup - AR disorder associated with defective renal and intestinal neutral amino acid transport, leading to reduced intestinal absorption of at least some neutral amino acids, most notably tryptophan - Most affected individuals, particularly those in high- income countries, remain asymptomatic throughout life, although symptoms can occur when certain exacerbating factors are present, e.g. poor nutrition or persistent diarrhea - When clinical features develop, the most common finding is a photosensitive "pellagra-like" dermatosis related to a relative niacin deficiency, as tryptophan is a precursor in niacin synthesis, in addition to ataxia and tremors - urine shows aminoaciduria - nicotinamide and high protein diet

with ataxia and tremor

IPEX syndrome - X-linked recessive disorder due to mutations in the FOXP3 gene that result in abnormal development of regulatory T cells. - present during infancy with severe diarrhea secondary to autoimmune enteropathy and develop a variety of autoimmune endocrinopathies (e.g. early-onset type 1 diabetes mel- litus, thyroiditis, cytopenias). - widespread eczematous and psoriasiform dermatitis and elevated IgE with frequent skin infections

with autoimmune diseases

sjogron's syndrome - autoimmune condition of the secretory glands presenting with xerostomia, xerophthalmia (Schirmer test) and arthritis - cutaneous get xerosis, purpura (palpable with vasculitis and non-palpable w/ capillaritis), urticarial vasculitis (hypo-complement) and annular erythema - risk for B cell lymphoma (MALT developing in glands); vasculitis, cryoglobulinemia and/or hypocomplemen- temia have poorer prognosis and inc risk of lymphoma - commonly have anti-SSA, SSB, elevated ESR, +RF, cryoglobulins - biopsy of minor salivary gland - emollients, artificial tears and saliva, humidifiers - steroids, MTX, rituximab reserved for vasculitis, neuropathy, or internal organ involvement

with dry eyes and mouth