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Pediatric emergency medicine trisk 1297 1297

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membrane zone First-line therapy is dapsone Other therapies include prednisone, sulfapyridine, colchicine, erythromycin, and topical steroids The condition tends to spontaneously remit over months to years but occasionally can be chronic Bullous Pemphigoid Bullous pemphigoid is a rare autoimmune blistering disorder that may develop as early as the newborn period and is due to autoantibodies to one of two hemidesmosomal proteins, BP 230 and BP 180, which help to maintain dermal– epidermal attachment Primary lesions include diffuse, large, tense, clear, or hemorrhagic bulla on a noninflammatory or erythematous base, although urticarial lesions occasionally predominate Common locations include forearms, abdomen, thighs, genitals, palms, and soles The striking acral involvement is more common in infancy and may help to distinguish clinically from other autoimmune blistering diseases Mucosal involvement may be seen Pruritus is common and may be severe Diagnosis is confirmed by biopsy of a vesicle demonstrating a subepidermal bulla with a predominantly eosinophilic infiltrate, and DIF from perilesional skin revealing linear deposition of immunoglobulin G (IgG) and C3 Enzyme-linked immunosorbent assay (ELISA) of BP 230 and 180 is commercially available as is indirect immunofluorescence testing for autoantibodies in the blood BP may also be initially confused with bullous impetigo The mainstay of treatment is oral corticosteroids, and the disease lasts an average of year Dermatitis Herpetiformis DH presents as symmetric, intensely pruritic, crusted papules, papulovesicles, and urticarial plaques overlying extensor surfaces of the elbows and knees, posterior neck, scalp, and buttocks Children with DH have gluten-sensitive enteropathy, however many are asymptomatic Only about 10% of patients with a diagnosis of celiac disease will present with the classic eruption of DH

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