Epidermolysis bullosa acquisita in childhood. Differentiation from hereditary epidermolysis bullosa.
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.
McCuaig
C C
CC
Department of Dermatology, University of Michigan Medical Center, Ann Arbor.
Chan
L S
LS
Woodley
D T
DT
Rasmussen
J E
JE
Cooper
K D
KD
D000367:Age Factors; D001706:Biopsy; D002648:Child; D003622:Dapsone / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D004820:Epidermolysis Bullosa / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D011241:Prednisone / Q000627:therapeutic use
D007074:Immunoglobulin G; D003622:Dapsone; D011241:Prednisone