Epidermolysis bullosa acquisita in childhood. Differentiation from hereditary epidermolysis bullosa
McCuaig, C.C.; Chan, L.S.; Woodley, D.T.; Rasmussen, J.E.; Cooper, K.D.
Archives of Dermatology 125(7): 944-949
1989
ISSN/ISBN: 0003-987X PMID: 2662910 Document Number: 328849
Epidermolysis bullosa acquisita (EBA), rarely reported in childhood, is described in a 10-year-old black girl. The age of onset during infancy and the clinical appearance mimicked hereditary dystrophic epidermolysis bullosa. Epidermolysis bullosa acquisita was diagnosed by direct immunofluorescence of perilesional skin, indirect immunofluorescence on normal epithelium and saline-split skin, direct immunoelectron microscopy, and immunoblotting of the patient's serum sample against partially purified EBA antigen/carboxyl domain of type VII collagen. Differentiation of mechanobullous disease in children is critical in that significant clinical benefit may be achieved in EBA with prednisone and/or dapsone therapy. A search for associated immunologic abnormalities and HLA-DR typing may help our understanding of EBA.