JOURNAL ARTICLE

[Linear IgA dermatosis in an adult with clinical signs of Stevens-Johnson syndrome]

B Schneck, C Termeer, M Mockenhaupt, M Augustin, E Schöpf
Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete 1999, 50 (4): 288-91
10354923
Immunohistologically linear IgA disease presents with unambiguous features, whereas clinical manifestations are variable. It sometimes shows similarity to other bullous dermatoses such as bullous pemphigoid and dermatitis herpetiformis. A 73 year old female patient was referred with the diagnosis of bullous pemphigoid. One day after admission clinical examination revealed the classical features of Stevens-Johnson syndrome (SJS): widespread confluent atypical target lesions, partly raised, partly flat with central blisters, and erythematous spots, but few typical targets, as well as blisters and large areas of skin detachment on her back and buttocks, accompanied by erosions of the oral and genital mucosa. Direct immunofluorescence performed on peri-lesional skin showed linear deposition of IgA along the basement membrane zone, leading to the diagnosis of linear IgA disease of adults. Our case report shows that linear IgA disease may present with the clinical pattern of SJS.

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