Familial epidermolysis bullosa acquisita.
Epidermolysis bullosa acquisita (EBA) is an acquired blistering skin disorder caused by IgG autoantibodies directed against type VII collagen. In contrast to the genetic forms of epidermolysis bullosa, EBA is usually an acquired, sporadic disease. In this report, we describe a family with two cases of EBA in an uncle-nephew pair, and a third family member with asymptomatic circulating anti-type VII collagen antibodies. These findings provide support for the hypothesis that there is a genetic component to EBA.
Noe
Megan H
MH
Department of Dermatology, University of Iowa, Iowa City, Iowa, USA.
Chen
Mei
M
Woodley
David T
DT
Fairley
Janet A
JA
D000369:Aged, 80 and over; D001323:Autoantibodies / Q000032:analysis*; D024143:Collagen Type VII / Q000276:immunology*; D004797:Enzyme-Linked Immunosorbent Assay; D016107:Epidermolysis Bullosa Acquisita / Q000235:genetics* / Q000276:immunology* / Q000473:pathology; D005260:Female; D019085:Fluorescent Antibody Technique, Direct; D006801:Humans; D008297:Male; D016253:Microscopy, Immunoelectron; D008875:Middle Aged; D012867:Skin / Q000276:immunology* / Q000473:pathology
D001323:Autoantibodies; D024143:Collagen Type VII