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Linear iga disease


Linear IgA Disease

General: Bullous dermatosis with pruritic urticarial 525b12f lesions with overlying vesicles or bullae; skin lesions heal without scarring; homogeneous deposition of immunoglobulin A (lgA) at the dermal-epidermal junction and, rarely, deposition of other immunoglobulin present; heterogeneous disease with regard to its clinical features, target antigens, and immunogenetics; association with HLA-B8, DR3, Cw7, and the linked rare tumor necrosis factor-α allele; may be induced by amiodarone.

Ocular: Chronic conjunctivitis; subconjunctival fibrosis; symblepharon; chronic progressive conjunctival cicatrization.

Clinical: Recurrent blistering skin disorder consisting of urticarial macules and plaques with vesicular eruptions on trunk and extremities; subepidermal vesiculation.

Aultbrinker EA, et al. Linear IgA disease. Ophthalmology 1988; 95:340-343.

Foster CS, et al. Immunosuppressive therapy for progressive ocular cicatricial pemphigoid. Ophthalmology 1982; 89: 341-353.

Primka EJ III, et al. Amiodarone-induced linear IgA disease. J Am Acad Dermatol 1994; 31:809-811.

Wojnarowska F, et al. Linear IgA disease: a heterogeneous disease. Dermatology 1994; 189[Suppl 1]:52-56.




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