[Linear IgA bullous dermatosis in children with autoantibodies against 180 kDa pemphigoid antigen]

F Bérard, J Kanitakis, M Di Maio, R Ghohestani, C Hermier, L David, A Claudy, M Faure
Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie 1996, 3 (4): 345-7

BACKGROUND: Linear IgA bullous dermatosis (LABD) is an autoimmune subepidermal blistering disease defined on the basis of direct immunofluorescence findings.

CASE REPORT: An 18 month-old girl suffering from LABD was studied by indirect immunofluorescence on salt-split skin and by Western blot in an attempt to characterize the involved autoantigen. Direct immunofluorescence showed an exclusive linear IgA deposit at the dermal-epidermal junction. Indirect immunofluorescence revealed circulating autoantibodies that reacted with the epidermal side of salt-split skin; they reacted by Western blot with a 180 kDa epidermal antigen, as in bullous pemphigoid.

CONCLUSION: This dermatosis fulfilling the clinical features and direct immunofluorescence criteria for childhood LABD seems to represent a case of IgA bullous pemphigoid. It further underscores the nosologic heterogeneity of LABD, which probably includes, apart from bullous pemphigoid, epidermolysis bullosa acquisita and cicatricial pemphigoid.

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