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Chronic Blistering Dermatoses Part 2

Chronic Blistering Dermatoses Part 2. David M. Bracciano, D.O. Pregnancy- Related Dermatoses. Intrahepatic Cholestasis of Pregnancy Polymorphic Eruption of Pregnancy Herpes (pemphigoid) gestationis purity Urticarial Papules and Plaques of Pregnancy (PUPPP) Papular Dermatitis of Pregnancy

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Chronic Blistering Dermatoses Part 2

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  1. Chronic Blistering Dermatoses Part 2 David M. Bracciano, D.O.

  2. Pregnancy- Related Dermatoses • Intrahepatic Cholestasis of Pregnancy • Polymorphic Eruption of Pregnancy • Herpes (pemphigoid) gestationis • purity Urticarial Papules and Plaques of Pregnancy (PUPPP) • Papular Dermatitis of Pregnancy • purity Folliculitis of Pregnancy

  3. Intrahepatic Cholestasis of Pregnancy • Generalized purities and jaundice • No primary skin lesions, secondary excoriations • Caused by cholestasis, occurs late in pregnancy, resolves after delivery • 0.5% of pregnancies • Tx; oral steroids

  4. Polymorphic Eruption of Pregnancy • Classification of all purity inflammatory dermatoses of pregnancy: • Toxemic rash of pregnancy • Pruigo annularis • EM gestationis • PUPPP • purity Folliculitis of Pregnancy

  5. Polymorphic Eruption of Pregnancy • Pruritic inflammatory dermatoses of pregnancy occur in 1 of every 120 to 240 • Treatment and prognosis is similar in subtypes

  6. Pruritic Urticarial Papules and Plaques of Pregnancy (PUPP) • First reported in 1979 • Erythematous papules and plaques that begin as 1-2 mm lesions within the abdominal striae • Spread over the course of a few days to involve the abdomen, buttocks, thighs • Upper chest, face, and mucous membranes spared

  7. PUPPP • Lesions coalesce to form urticarial plaques • Intense pruritis is characteristic • Primigravidas 75% of the time, usually does not recur with subsequent pregnancies • Begins late in third trimester and resolves with delivery • May be associated with increase weight gain

  8. PUPPP • Histology: perivascular infiltrate in upper and mid dermis, epidermis normal • Tx: topical or oral steroids

  9. Papular Dermatitis of Pregnancy • Pruritic generalized eruption of 3-5 mm erythematous papule surmounted by a small, firm, central crust • May erupt at any time during pregnancy and resolve with delivery • Marked elevation of urine HCG • Tx; oral steroids, may recur in subsequent pregnancies

  10. Prurigo Gestationis (Besnier) • purity, excoriated papules of the proximal limbs and upper trunk • Onset is 20-34 weeks gestation • Clears in postpartum period and does not recur • Tx: topical steroids

  11. Pruritic Folliculitis of Pregnancy • 2nd or 3rd trimester • Small follicular pustules scattered widely over the trunk • May be a type of hormonally induced acne

  12. Impetigo Herpetiformis • Form of severe pustular psoriasis occurring in pregnancy • Acute, usually febrile onset of grouped pustules on an erythematous base • Begins in the groin, axillae, and neck • Increased WBC, hypocalcemia • Recurs with pregnancy, fetal death due to placental insufficiency • Tx; prednisone 1mg/kg

  13. Cicatricial Pemphigoid (Benign Mucosal Pemphigoid) • Vesicles which quickly rupture, leaving erosions and ulcers with scarring • Primarily occur on mucous membranes, conjunctiva (66%) and oral mucosa (90%) • Oral mucosa may be the only affected site for years; desquamative gingivitis of buccal mucosa

  14. Cicatricial Pemphigoid • Tends to affect middle-aged to elderly women 2:1 female/male • Ddx; oral lichen planus (biopsy and IF) • Chronic disease that may lead to slowly progressive shrinkage of the ocular mucous membranes and blindness • Also occurs in pharynx, esophagus, larynx, nose, penis, vagina, anal mucosa, deafness

  15. Cicatricial Pemphigoid • Cutaneous lesions in 25%; tense bullae • Bullae heal with or without scarring, occur on the face, scalp, neck, and inguinal region and extremities • Some pts may have antibodies targeted against classic bullous pemphigoid antigens and should be classified as “mucosal predominate bullous pemphigoid” • Chronic course, pts health not usually affected • IgA antibodies may explain mucosal scarring tendency

  16. Cicatricial Pemphigoid • Little tendency to remission (unlike bullous pemphigoid) • Subtypes include types that target basement membrane zone antigens (laminin, glycoproteins, )

  17. Cicatricial Pemphigoid • Direct IF testing C3 and IgG at the lamina lucida in 80-95% • Tx: mild cases topical steroids (Temovate/Orabase), intralesional triamcinolone every 2-4 weeks • Tx: Dapsone, prednisone, Azathioprine or cyclophosphamide

  18. Epidermolysis Bullosa Acquisita • Antibodies to Type VII collagen • Skin fragility, healing with scars • Bullous eruption, scaring, milia • Need to exclude all other bullous diseases: porphyria cutanea tarda, pemphigoid, pemphigus, dermatitis herpetiformis, and bullous drug eruption

  19. Epidermolysis Bullosa Acquisita • Tx; unsatisfactory, steroids, dapsone, colchicine, IV Immunoglobulin, Cyclosporin

  20. Dermatitis Herpetiformis • Chronic, relapsing, severely purity disease • Grouped symmetrical, polymorphous, erythematous-based lesions • May be papular, papulovesicular, vesiculobullous, bullous, or urticarial • Itching and burning are intense • Spontaneous remissions lasting a week

  21. Dermatitis Herpetiformis • Eruption usually symmetrical • Scalp, nuchal area, posterior axillary folds, sacral region, buttocks, knees, forearms • Pruriginous papules are a common feature • Vesicles are more common than bullae; however all types of these lesions may be present in one patient • Course of the disease is generally lifelong, with prolonged remissions being rare

  22. Dermatitis Herpetiformis • Very few patients with DH ever have diarrhea although DH is associated with Gluten-sensitive-enteropathy (GSE) • 87% of pts with DH and IgA deposits in the skin are HLA-B8 positive (like GSE) • Gluten is a protein found in cereals except for rice, oats, and corn • IgA antibodies are formed in the jejunum, may deposit in the skin

  23. Dermatitis Herpetiformis • Associated with; Thyroid disorders, small bowel lymphoma, non-Hodgkins lymphoma • 70% of pts have abnormalities of the jejunal mucosa • Gluten-free diet decreases Dapsone dose requirements after 3-4 months

  24. Dermatitis Herpetiformis • Ddx: pemphigoid, EM, scabies, contact dermatitis, atopic dermatitis, eczema, insect bites, pruigo nodularis • IgA in a granular pattern in the dermal papillae in normal skin is specific and pathognomonic for DH

  25. Dermatitis Herpetiformis • IgA deposits may be focal, so multiple biopsies may be needed. • Deposits of the antibody are more often seen in previously involved skin or normal appearing skin adjacent to involved skin

  26. Dermatitis Herpetiformis • Equal male:female • Onset between 20 to 40 years • Tx: Dapsone 50-300mg daily (hemolytic anemia, methemoglobinemia, check G6PD prior to tx) monitor Hct,WBCs, LFTs • Tx: Sulfapyridine 0.5g QID to 2-4g/day • Gluten-free diet will decrease need for meds or allow pt to go off them Celiac Society

  27. Linear IgA Bullous Dermatosis • Subepidermal blisters, a neutrophillic infiltrate, circulating IGA antibasement membrane zone antibody • Deposition of IgA antibody at the dermoepidermal junction by direct IF

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