Pemphigoid. Bullous and cicatricial. 1990

G J Anhalt
Department of Dermatology, Johns Hopkins University, Baltimore, Maryland.

Bullous pemphigoid is a blistering disease of the elderly that often is self-limited. It is apparently caused by autoantibodies against antigens of the basal cell hemidesmosome. There is apparently more than one antigen, but the most significant has a molecular weight of about 230 kD and the second most frequently detected antigen has a lower molecular weight of about 166 to 180 kD. The subepidermal blister presumably is produced by autoantibody binding along the basement membrane zone, followed by a complex series of inflammatory events. Conservative use of oral and topical corticosteroids often is sufficient to control the disease. Cicatricial pemphigoid has similar immunologic features, but it is not known if the same antigens are immunogenic in this disease. Scarring of mucosal surfaces due to recurrent blistering can result in blindness, asphyxiation, and esophageal stenosis. Treatment is more difficult and often requires short-term, intensive immunosuppression with cyclophosphamide.

UI MeSH Term Description Entries
D008297 Male Males
D010390 Pemphigoid, Benign Mucous Membrane A chronic blistering disease with predilection for mucous membranes and less frequently the skin, and with a tendency to scarring. It is sometimes called ocular pemphigoid because of conjunctival mucous membrane involvement. Pemphigoid, Cicatricial,Benign Mucosal Pemphigoid,Benign Mucous Membrane Pemphigoid,Cicatricial Pemphigoid, Ocular,Mucous Membrane Pemphigoid, Benign,Ocular Cicatricial Pemphigoid,Pemphigoid, Ocular Cicatricial,Benign Mucosal Pemphigoids,Cicatricial Pemphigoid,Cicatricial Pemphigoids, Ocular,Mucosal Pemphigoid, Benign,Mucosal Pemphigoids, Benign,Ocular Cicatricial Pemphigoids,Pemphigoid, Benign Mucosal,Pemphigoids, Benign Mucosal,Pemphigoids, Ocular Cicatricial
D010391 Pemphigoid, Bullous A chronic and relatively benign subepidermal blistering disease usually of the elderly and without histopathologic acantholysis. Pemphigoid,Bullous Pemphigoid,Pemphigoids
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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