Bordier-Lamy F, Eschard C, Coste M, Ploton D, Durlach A, Tabary T, Bernard P
[Epidermolysis bullosa acquisita of childhood.] [English Abstract, Journal Article]
Ann Dermatol Venereol 2009 Jun-Jul; 136(6-7):513-7.
BACKGROUND: Epidermolysis bullosa acquisita (EBA) is a subepidermal autoimmune blistering disease characterized immunologically by autoantibodies to type VII collagen. Its occurrence in childhood is rare. Thirty-five cases have been described to date in the literature.
PATIENTS AND METHODS: We report the case of an 8-year-old girl presenting blistering lesions on the cheeks, extremities and limb extension areas. The diagnosis of EBA was confirmed by histology, direct immunofluorescence of a perilesional skin biopsy specimen, indirect immunofluorescence on salt-split skin substrate and direct electron microscopy. The patient was controlled clinically under treatment with dapsone alone.
DISCUSSION: This 36th childhood case of EBA presented typical clinical features, a similar prognosis and comparable treatment response to other paediatric cases. Clinical presentation is inflammatory and affects the face. As in our case, in childhood, prognosis is often better than in adults without the need for immunosuppressive agents.
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