A case of an inflammatory variant of epidermolysis bullosa acquisita: chronic bullous dermatosis associated with nonscarring mucosal blisters and circulating IgG anti-type-VII-collagen antibody.
A 42-year-old man showed prominent blistering lesions of the mouth and esophagus in addition to a few bullous lesions of the skin. Direct immunofluorescence microscopy revealed distinct linear deposition of IgG and C3 at the epidermal basement membrane zone where slight deposition of IgA and IgM was also observed. In direct immunoelectron-microscopic examination, antibody was detected in the sublamina densa of the basement membrane zone. Immunoblot analysis with dermal extracts demonstrated that the patient's serum contained circulating IgG antibodies against the 290-kD protein, which comigrated with type VII collagen. The lesions healed without any scars. The results of these studies corresponded to the laboratory findings in epidermolysis bullosa acquisita (EBA), although the clinical features were distinct from classic EBA.
Tokuda
Y
Y
Department of Dermatology, Shinshu University School of Medicine, Matsumoto, Japan.
Amagai
M
M
Yaoita
H
H
Kawachi
S
S
Ito
T
T
Matsuyama
I
I
Tsuchiya
S
S
Saida
T
T
D000328:Adult; D000906:Antibodies / Q000097:blood; D001768:Blister / Q000209:etiology; D002908:Chronic Disease; D003094:Collagen / Q000276:immunology*; D003937:Diagnosis, Differential; D016107:Epidermolysis Bullosa Acquisita / Q000150:complications / Q000175:diagnosis* / Q000276:immunology* / Q000473:pathology; D004945:Esophagoscopy; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008297:Male; D008856:Microscopy, Fluorescence; D009092:Mucous Membrane / Q000473:pathology
D000906:Antibodies; D007074:Immunoglobulin G; D003094:Collagen