title: Familial epidermolysis bullosa acquisita.
abstract: 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.
authors.affiliation: Department of Dermatology, University of Iowa, Iowa City, Iowa, USA.
authors.forename: Megan H
authors.initials: MH
authors.lastname: Noe
authors.forename: Mei
authors.initials: M
authors.lastname: Chen
authors.forename: David T
authors.initials: DT
authors.lastname: Woodley
authors.forename: Janet A
authors.initials: JA
authors.lastname: Fairley
mesh_terms: 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
chemical_list: D001323:Autoantibodies; D024143:Collagen Type VII