Allergic and immune-related disorders Atopic dermatitis (Eczema) Relapsing inflammatory skin disorder Pruritis (*Intense itching) Lichenification

Commonly associated w/ asthma + allergic rhinitis Triggers: Climate, food, contact w/ allergens, physical/chemical irritants, emotional factors Contact (including hand) dermatitis Type IV hypersensitivity rxn from contact w/ allergen pt was previously exposed and sensitized to Pruritis, rash Frequent allergens: poison ivy, poison oak, nickel, soaps, detergents, cosmetics, rubber products containing latex (gloves, elastic bands in clothing) Pathogenesis: Allergens passed through epidermis taken up by langerhans cells carried to LNs exposed to T lymphocytes Common in infancy Infants: Erythematous, weeping, pruritic patches on face, scalp, diaper area Children: Dry, scaly, pruritic, excoriated patches in flexural areas + neck Adults: Lichenification and dry, fissured skin, often limited to hands Distributions where makeup, clothing, perfume, nickel jewelry, and pants contact skin Begins in area of contact w/ antigen Acute: 24-48 hrs after contact. Erythematous tiny blisters scale + crusts. Intense pruritis Subacute: episodic exposure or weak allergen. Chronic: extended exposure. Erythema, lichenification w/ fissuring, often w/ superimposed acute dermatitis More common in Adults Sebhorrheic dermatitis Inflammatory papulosquamous dz associated w/ sebaceous follicle regions Related to abnormal immune response to Malassezia (Pityrosporum) common skin yeast **Pts w/ HIV/AIDS can develop severe seborrheic dermatitis or overlapping syndrome *Suspect HIV in young person w/ severe seborrheic dermatitis Psoriasis T-cell mediated inflammatory dermatosis dermal inflammation and epidermal hyperplasia Well-defined pink to erythematous patches covered with silvery scales, revealing small bleeding points on scraping (Auspitz sign) 5% have also have seronegative arthritis Psoriatic nails: pitting, oil spots, and onycholysis (lifting of nail plate) Provoked by: Local irritation, trauma (Koebners phenomenon) Step infxn cutaneous immune complex deposition triggers guttate B-blockers, lithium, ACEIs Psoriatic arthritis: Begins on hands (sausage digits) Pts w/ spinal involvement = HLA-B27 + Pustular psoriasis (less common) Life-threatening when generalized Fever, electrolyte abnormalities, loss of serum proteins Urticaria (Hives) Type I hypersensitivity response: Mast cells release vasoactive substances (histamine, protaglandins) Acute: response to trigger (food, drug, virus, insect bite, physical stimulus) Chronic: Idiopathic Superficial, intense edema in localized area Elevated papules or plaque reddish or white, variable size. Widespread, last a few hours. Severe rxs extracutaneous manifestations: Tongue swelling, angiodema (deeper, more diffuse swelling), asthma, GI sx, joint swelling, fever Eruptions: 7-14 days after exposure If pts reascts w/in 1-2 days of starting new drug (i.e. never taken before) that drug not likely causative agent Widespread, relatively symmetrical, + pruritic Clinical impression Histo: eosinophils D/c offention agent Symptoms: Antihistamines Clinical Biopsy: perivascular edema Systemic antihistamines *Topical meds = NO benefit Areas w/ oily skin scalp, eyebrows, nasolabial folds, midchest Infants: severe, red diapher rash w/ yellow scale, erosions, + blisters. Thick crust (cradle cap) seen on scalp. Children/Adults: Red, scaly patches around ears, eyebrows, nasolabial fold, midchest, scalp Starts in puberty or young adulthood Extensor surfaces: elbows, knees, lumbosacral regions Initially very small (guttate) May slowly enlarge and become confluent Clinical Biopsy: thickened epidermis, elongated rete ridges, absent granular layer, preserved nuclei, and sterile neutrophilic infiltrate (munros microabscess) in stratum corneum Topical steroids + keratolytic agents, tar, or anthralin + UV therapy (PUVA) Severe: Methotrexate Arthritis: NSAIDs Methotrexate *Avoid systemic steroids (tapering flares) Clinical r/o contact dermatitis and psoriasis Scalp: Selenium Sulfide Zinc pyrithione shampoos Other areas: Topical antifungals Topical corticosteroids Clinical Possible eosinophils and IgE r/o seborrheic dermatitis, contact dermatitis, pityriasis rosea, drug eruption, and CTCL Clinical Patch test Differential: Atopic dermatitis seborrheic dermatitis Impetigo HSV Herpes zoster Fungal infection Topical or systemic corticosteroids Prophylaxis: Moisturizers Nondrying soaps (Dove) Avoid triggers Topical steroids

Prophylaxis: Avoid allergen

Drug Eruption

Maintain high suspicion in hospitalized pts who develop rash Can cause all four types of hypersensitivity rxns

 Short-lived (gone 1-2 weeks after cessation) Exception: Fixed drug eruption Reddish macules/papules that develop in same area (genitalia, face, extremities) each tim pt is exposed to triggering agent When lesions resolve brown pigmentation Extreme complications: erythroderma and TEN Erythema multiforme Cutaneous rxn pattern w/ classic targetoid lesions w/ many triggers. Often recurrent. Triggers: Recurrent HSV infxn of lip, drugs (sulfas, anticonvulsants, barbs, penicillin, NSAIDs), mycoplasmal infections Target appearance (erythematous macules centrally cleared blister) Palms + soles affected Possible systemic sx (Fever, myalgias, HA, arthralgias) Minor form: Uncomplication, localized to skin Severe: Can TEN or SJS (Pts very ill, involved at least 2 mucosal surfaces) StevensJohnson Syndrome (SJS)/Toxic Epidermal Necrolysis (TEN) Life-threatening exfoliative mucocutaneous dz often caused by drug0induced immunologic rxn SJS: epidermal separation <10% BSA TEN: >30% BSA Assoc. w/ hx of new drug Penicillin, Sulfonamides, Qunilones, Cephalosporins Seizure meds (phenytoin, carbamazepin) Allopurinol Corticosteroids NSAIDs Erythema Nodosum Panniculitis (inflammation of fat cells under skin) Triggers: Infection (Strep, Coccidoides, Yersinia, TB) Drug rxns (Sulfas, various antibiotics, OCPs) Chonic inflammatory diseases (Sarcoid, Crohns, UC, Behcets) **Pts may have false-+ VDRL (as in SLE) Pemphigus vulgaris *Involves mucous membranes Life-threatening autoimmune condition w/ intraepidermal blister widespread painful erosions of skin and mucous membranes Abs against desmoglein molecules responsible for keratinocyte adherence loss of cellular attachment Middle-aged 40-60 Initial: mucous membrane involvement (mouth ulcers) w/ progression to skin Severe mucosal erosions w/ widespread erythematous, cutaneous macules or atypical targetoid lesions Epidermal lesions become confluent, + Nikolskys sign, epidermal detachment Mucous membranes of eyes, mouth, and genitals often become eroded and hemorrhagic Painful, erythematous nodules on pts lower legs Slowly spread turning brown or grey May present w/ fever and joint pain TEN: full-thickness eosinophilic epidermal necrosis Possibley preceded by EM (Flu-like prodrome, skin tenderness, maculopapular drug rash, or painful mouth lesions) BIOPSY: SJS: degeneration of basal layer of epidermis Pts have same complications as burn victims (Thermoregulatory difficulties, electrolyte disturbances, 2 infxns) Tx: Skin coverage Maintenance of fluid + electrolyte balance High risk of mortality Clinical Hx of recurrent labial herpes Should be sought in all cases w/ multiple recurrences Symptomatic tx Minor: antipruritics Major: Treat as burns Pts w/ HSV: suppressive acyclovir may frequency of rashes *Corticosteroids no benefit

Diff: Graft-vs-host rxn (after BM transplant) Radiation therapy Burns Clinical Histo: nonspecific panniculitis Workup: ASO titer PPD test (in high risk) CXR (r/o sarcoid) Small bowel series (r/o IBD in pts w/ GI sx) Clinical + Nikolskys sign (produce blister by rubbing skin adjacent to natural blister) Skin bx w/ IF: Acantholysis (intraepidermal split w/ freefloating keratinocytes in blister) IF + ELISA = confirmatory for antidesmoglein Abs

Remove triggering factor and treat underlying dz NSAIDs can be used but may EM

Long term tx required Intital: Systemic corticosterois at high doses Steroid-sparing agents introduced early to steroid SEs (mycophenolate mofetil and azathioprine)

Bullous (and cicatricial) pemphigoid

Chronic, acquired autoimmune blistering dz w/ Abs against hemidesmosomal Ag separation at epidermal BM MC: 60-80 yo Pathogenesis: Abs agains bullous pemphigoid Ag (lies superficially in BM zone (BMZ)) Ag-Ab complexes activate complement + eosinophil degranulation inflammation rxn separation at BMZ

Blisters are stable d/t nearly normal epidermis roof Firm, stable blister on erythematous skin Often preceded by uriticarial lesion - Nikolskys sign Form crusts + erosions Mucous membranes less commonly involved than in pemphigus

Clinical Biopsy: subepidermal blister, w/ eosinophil-rich infiltrate IF Linear IgG + C3 Ig + Complement at dermalepidermal junction

Systemic corticosteroids Topical steroids can help prevent blister formation when applied to early lesions