Urticaria & Hypersensitivity Syndromes

How long till the lesions in erythema multiforme syndrome heal?

1-2 wks w/o scaring

When do onset of sx occur in SJS/TEN?

1-3 wks post drug exposure

What is the incidence rate of angioedema?

15-25% of pts w/urticaria 10% alone 50% will also have urticaria

What is the peak incidence of erythema nodosum?

20-30 yo F>M (3-6:1)

What is the age of onset for Behcet's Syndrome?

3rd and 4th decades of life

What population is mostly affected by erythema multiforme syndrome?

50% of pts < 20yo M>F

What is the duration of acute urticaria?

<6 wks

What is the duration of chronic urticaria?

>6 wks

What are the major criteria for Sweet's Syndrome?

Abrupt onset of typical cutaneous lesions Histopathology consistent with Sweet's Syndrome

Sweet's Syndrome is also know as?

Acute Febrile Neutrophilic Dermatosis

What underlying disorders are associated with PG?

Age distribution reflect major disorders IBD Inflammatory arthritis Plasma cell dyscarsias Other hematologic malignancies

What disorder is characterized as an acute or chronic hive like swelling in the subcutaneous tissue of the skin and mucosa?

Angioedema

How would you determine interstitial lung dz in regards to patients with dermatomyositis/polymyositis?

Baseline PFTs High-resolution CT may be required to detect involvement

What condition has a highly variable coarse w/recurrences and remissions and higher mortality rate w/CNS involvement?

Behcet's Syndrome

What condition may also have synovitis, neurologic disorders and thrombophlebitis?

Behcet's Syndrome

What is one of the leading causes of blindness in Mediterranean and East Asians with severe cases?

Behcet's Syndrome

What condition is characterized as a multi-system vasculitic inflammation and hallmark is recurrent oral pathos ulcers and any of the 2 following: recurrent genital aphthous ulcers, anterior or posterior uveitis, erythema nodosum or pustules, + pathergy test?

Behcet's syndrome

How much criteria is required for the dx of Sweet's Syndrome?

Both major and two major

In Sweet's Syndrome, the lesions can become what if edema is intense?

Bullous

What is the DDx for Sweet's Syndrome?

Bullous pyoderma gangrenosum, EM, cellulitis, vasculitis

What is the Ddx for erythema nodosum?

Cellulitis Infected insect bites Minor trauma Nodular vasculitis HSP Erythema induratum Other forms of panniculitis

What is the physically prevention of erythema multiforme syndrome?

Classic target (iris) lesion results from spread of red maculopapule to 1-3 cm as center becomes cyanotic, purpuric or vesicular Bullae and erosions may appear in oral area

What are the s/s in the presentation of angioedema?

Deeper run produces more diffuse swelling Lips, palms, soles, limbs, trunk and genitalia are most commonly affected GI/Resp tract produces dysphagia, dyspnea, colicky abd pain, & attaches of V/D Itching usually absent May have burning and painful swelling

Gottron's papules and Gottron's sign are associated with what disorder

Dermatomyositis/Polymyositis

What disorder is considered an autoimmune connective tissue dz that may be triggered by malignancy, drugs, or infectious agents and has a midomal age distribution with female predominance?

Dermatomyositis/Polymyositis

What is the pathogeneses of dermatomyositis and polymyositis?

Different Humoral immunity vs cell-mediated immunity

What are the histological findings in Sweet's Syndrome?

Diffuse infiltrates of neutrophils within dermis and occasionally subcutaneous fat

What is the DDX for erythema multiforme syndrome?

Drug eruption Psoriasis Secondary syphilis Urticaria

What is the DDX in regards to urticaria?

Drug eruption Viral exanthem Bites bulls pemphigoid in an elderly patient Urticaria vasculitis in lesions lasting >24hrs Hereditary angioedema

What is the most common etiology of urticaria?

Drugs, food and infection

What disorder is relatively common, acute, and often recurrent inflammatory dz that has a reaction pattern of blood vessels in the dermis w/secondary epidermal changes with cutaneous lesions that target lesions and urticarial plaques in a symmetric pattern on dorsal of hands, feet and extensor surfaces of forearms and legs?

Erythema Multiforme Syndrom

Urticarial plaques that are the same size and remain unchanged for days and may occur w/o target lesions in same distribution are associated with what disorder?

Erythema Multiforme Syndrome

What disorder has painful red nodules on lower legs that are bilateral but not symmetric and lesions may also occur on knees and arms but rarely on face and neck and can have fatigue, malaise, and arthralgia or sx of upper respiratory infection precede eruption by 1--3 wks?

Erythema Nodosum

What disorder is characterized by acute inflammatory/immunologic reaction of the SQ fat?

Erythema Nodosum

What is the clinical presentation of urticaria?

Erythematous, non pitting, edematous plaques that change in size and shape by peripheral extension and regression and new lesions evolve as old ones resolve and may become confluent.

Prevalence rate of urticaria?

Extremely common Est 15-25% of pop during their life

What are the systemic findings associated with Behcet's Syndrome?

Eyes Nonerosive asymmetric oligoarthritis Meningoencephalitis, benign inter cranial HTN, cranial nerve palsies, brainstem lesions, psychosis Aneurysms, venous thrombosis, coronary vasculitis Aphthous ulcers thru out GI tract

Where else can ulcers appear besides the lower extremities in pyoderma gangrenosum?

Face, upper extremities and trunk

What are the associated systemic findings in Sweet's Syndrome?

Fever, malaise, and arthralgia, and peripheral leukocytosis Manifestations may develop including ocular, pulmonary, and skeletal involvement

What are some etiology of acute angioedema?

Food, drugs, contrast dye, stinging insect venom, and pollen

Etiology of Urticaria (hives)

Foods and additives Drugs Infections Inhalants Internal Dz Physical stimuli Non-immunologic contact urticaria Hormones (prego) Skin Dz Genetics (rare)

What are the minor criteria for PG?

Hx suggestive of potherb or clinical finding of cribriform scarring Systemic disease assoc w/PG Histopathologic findings (sterile dermal neutrophilic, +/- mixed inflammation, +/- lymphocytic vasculitis Tx response (rapid response to systemic corticosteroids)

What is the cause of 50% of Sweet's Syndrome

Idiopathic

What are the underlying disorders associated with Sweet's Syndrome?

Infections Hematologic malignancies IBD Drugs Carcinomas

What causes erythema nodosum?

Infections, drugs, other inflammatory/grannulomatous dz (sarcoidosis)

Typical symptoms of dermatomyositis/polymyositis?

Inflammatory myopathy Difficulty combing hair or rising from sitting position

How can lesions begin in regards to pyoderma gangrenosum?

Inflammatory papulopustule (which may be follicular), as a bulla on violaceous base, or at site of trauma (pathergy)

What histopathology changes occur with dermatomyositis/polymyositis?

Interface dermatitis of skin with mucin; lymphocytic myositis in affected muscle Autoantibodies numerous

What are the assoc s/s of urticaria?

Itching with various intensity Pruritus is milder in deep hives (angiodema)

What population has the highest prevalence of Behcet's Syndrome?

Japan, SE Asia, Middle East, Southern Europe

What is the mean age for dermatomyositis/polymyositis?

Juvenile 8yo Adult 52 yo

What would the skin bx show in regards to Behcet's Syndrome?

Leukocytoclastic vasculitis in acute early lesions Lymphocytic vasculitis in late lesions

Where does angioedema usually affect?

Lips, palms, soles, limbs, and genitalia

What is the most common trigger for dermatomyositis/polymyositis?

Malignancy (breast CA, ovarian CA)

What is the etiology go acquired C1q esterase inhibit deficiency in regards to chronic angioedema

Malignancy (B-cell lymphoma and breast CA) and Autoimmune form

How would the central region of utericaria present?

May clear producing an annular pattern

What population is association with Behcet's Syndrome?

Mediterranean/East Asia have HLA assoc USA and Europe do not Male > Females (dependent on ethnic background)

What is the treatment course for erythema multiforme?

Mild cases not tx Pts w/many target lesions respond rapidly to prednisone 1-3wks course, and can abort a recurrence Oral acyclovir/valacyclovir to prevent HSV induced recurrent EM

How would SJS/TEN skin lesions be described as?

Morbilliform Early: Macular areas w/crinkled surface that enlarge and coalesce Raised flaccid blisters that spread w/lateral pressure (Nikolsky sign) on erythematous base W/trauma-filled thickness epidermal detachment resembling a 2nd degree burn

What is the etiology of erythema multiforme syndrome?

More than 50% idiopathic Drugs: sulfonamides, PCN, allopurinol, phenytoin, barbiturates Infections: esp following herpes simplex, mycoplasma

Prognosis of urticaria/angioedema?

Most do well Acute forms resolve when offending agent removed Chronic - prophylactic txs helpful

What is the etiology of chronic angioedema?

Most idiopathic F>M 40-50 yo GI/Resp tract may be involved (asphyxiation no danger) Acquired C1q esterase inhibitor deficiency Hereditary angioedema

What population is Sweet's Syndrome seen in?

Most often in adults Female:Male ration 4:1 May first appear or flare during pregnancy

What drugs could cause acute angioedema?

NSAIDS, ASA, ACE inhibitors

Diagnosis of urticaria?

No routine labs Skin findings HX (time of onset, foods, meds, exposure) Physical exam (r/o infection & internal dz) Skin bx if necessary (chronic) R/o physical urticaria

What is a less common symptom of Sweet's Syndrome?

Nodules develop due to neutrophilic panniculitis or pustules with plaques

What drugs may illicit urticaria?

PCN, ASA, Sulfa, morphine, etc

What are erythema nodosum-like lesions?

Painful inflammatory nodules on arms and legs found in Behcet's Syndrome

What are the skin findings found in Behcet's Syndrome?

Painful oral ulcers erupt in cyclic fashion and may persist/recur for wks to months before other sx appear Aphthous ulcers on oral or genital mucosa Erythema nodosum-like lesions Inflammatory pustules, thrombophlebitis, inflammatory plaques, pyoderma gangrenosum-like lesions palpable purpuric lesions

What is the most common presentation of pyoderma gangrenosum?

Painful, rapidly enlarging ulcer of lower extremity with gray-violet, undermined necrotic border Often peripheral rim of erythema and base of ulcer may be purulent

What is the systemic findings in regards to the eyes in Behcet's Syndrome?

Posterior or anterior uveitis, retinal vasculitis, hypopyon, secondary cataracts, glaucoma, neovascular lesions

What are the minor criteria of Sweet's Syndrome

Preceded by one fo the aspic infections or vaccinations; accompanied by one of the aspic malignancies or inflammatory disorders; assoc with drug exposure or pregnancy Presence of fever and constitutional s/s Leukocytosis Excellent response to systemic corticosteroids

What is the major criteria for dx of PG?

Rapid progression of painful, necrolytic cutaneous ulcer with an irregular violaceous and undermined border Other causes of cutaneous ulceration have been excluded

What is the treatment options for urticaria?

Remove offending agent Antihistamines Prednisone for nonresponse to antihistamines Epi for extensive/severe cases Keep pt cool physically and emotionally

What is required for the dx of PG?

Requires both major and at least two minor criteria

What disorder has mild to moderate skin tenderness, conjunctival burning or itching, than skin pain, burning, tenderness and paresthesia and mouth lesions are painful and they may have photophobia, painful urination and anxiety?

SJS/TEN hx

What is the DDX for dermatomyositis/polymyositis?

SLE, psoriasis, contact dermatosis, photo-drug eruption, CTCL, atopic dermatitis, systemic sclerosis, etc

What internal diseases may cause urticaria?

SLE, thyroid (hyper/autoimmune), etc

How is angioedema diagnosed?

Same as urticaria Serum levels for C1 inhibitor for chronic Thyroid antibodies present in some chronic patients

What foods may illicit urticaria?

Shellfish, nuts, eggs, chocolate, strawberries, milk, etc Food additives

What is the treatment for Sweet's Syndrome?

Spontaneously resolves over few months May recur in 30-50% with idiopathic vs malignancy-associated Antimicrobials for underlying infections Systemic corticosteroids, dapsone, etc.

What disorder is characterized by mucocutaneous drug-induced or idiopathic run patterns with skin tenderness and erythema of skin and mucosa, followed by extensive cutaneous and mucosal epidermal necrosis and sloughing?

Steven-Johnson Syndrome/Toxic Epidermal Necrolysis

What is the treatment plan for drug induced rxn?

Stop drug Urticarua/angioedema as before Anaphylaxis: SQ EPI, IM antihistamines, aminophylline, fluids Oral/IV glucocorticoids depending on severity Topical steroids in some instances Oral antihistamines

Acute onset of erythematous edematous papules and plaques that are tender, but not pruritic that typically occur on face, neck, upper trunk, and upper extremities?

Sweet's Syndrome

What is the recommended treatment for erythema nodosum?

Symptomatic relief: self-limited dz NSAIDS (indomethacin) Compressive bandages and bed rest Oral corticosteroids seldom necessary, but response is rapid

What is the treatment for dermatomyositis/polymyositis?

Systemic corticosteroids IVIG Treat underlying malignancy

Fever is usually higher in: SJS or TEN

TEN

What is the lab work-up for erythema nodosum?

Throat culture ASO titer ESR CXR (r/o sarcoidosis) PPD -RF

What are the treatment options for Behcet's Syndrome?

Topical steroids, ILS, thalidomide, colchicine and dapsone for oral ulcers Prednisone w/or w/o azathioprine, cyclosphamide, cyclosporine, azathioprine alone

What is the DDX for Behcet's Syndrome?

Ulcers: herpetic infection hand-foot-mouth-dz, varicella-zoster, chancre, SCC

What are the treatment options for PG?

Underlying disorder Systemic corticosteroids ILS Cyclosporine TNF alpha inhibitors Topical tacrolimus Minocycline

What is the etiology of Behcet's Syndrome?

Unknown

Condition that is a vascular reaction characterized by wheals, white or red evanescent plaques, generally surrounded by a halo or flare and usually assoc w/severe itching, stinging, or pricking sensations?

Urticaria

What are the characteristic cutaneous findings in dermatomyositis/polymyositis?

Violaceous hue of the upper eyelids with periorbital edema (heliotrope sign) Nail fold telangiectasia

What are the treatment options for chronic angioedema?

antihistamines and corticosteroids C1 deficiencies: androgens, antifibrinolytics and fresh plasma/purified C1 inhibitor

What infections may cause urticaria?

chronic bacterial infections, viral infections, protozoal and helminth

How long does erythema nodosum last?

condition may recur for months or years and is typically benign in most pts

What is the DDX of angioedema?

consider contact dermatitis

What is polymyositis?

disease that affects muscle only

Healing of PG ulcers often leads what type of scarring?

distinctive cribriform pattern

What is the distribution of the plaques in urticaria?

haphazard

How do new lesions appear in erythema multiforme syndrome?

in crops

What system disease affects 15-30% of dermatomyositis/polymyositis patients?

interstitial lung disease

What is the benefit of using topical steroids in the treatment of urticaria?

little

In regards to urticaria, wheals are caused by what?

localized edema

SJS is considered?

maximal variant of EM (major)

TEN is considered?

maximal variant of SJS

Where on the body would erythema multiforme syndrome present?

on dorsal of hands, feet and extensor surface of forearms and legs

How often is the cause found in chronic urticaria?

only about 5-20% of pts

What inhalants may cause urticaria?

pollen, dust mite, animal dander

What would a skin bx show in erythema nodosum?

reveals septal panniculitis

What is the etiology of acute angioedema?

severe allergic type IgE mediated hypersensitivity reaction

What is the most characteristic finding of erythema multiforme syndrome?

target lesions and papules that are dusky red, round maculopapules suddenly appearing in symmetric pattern

SJS and TEN both start with what type of lesions?

target like lesions however 50% of TEN cases do not

What is the treatment options for acute or severe angioedema?

w/epi and high dose antihistamines

When do bullae or purpuric lesions appear in regards to urticaria presentation?

with intense swelling