Localized linear IgA/IgG bullous dermatosis.
Linear IgA/IgG bullous dermatosis (LAGBD) is an auto-immune blistering disease characterized by the local accumulation of IgA- and IgG-class anti-basement membrane autoantibodies. It typically presents as a generalized pruritic vesiculobullous eruption. No cases of localized LAGBD have yet been reported. We report a case of a 78-year-old man with LAGBD localized to the perianal area. The patient complained of suffering from persistent ulcers around the anus for more than 3 years. Physical examination revealed several blisters and ulcers up to 2-cm in diameter around the anus. No lesions were found elsewhere on the body. Histological analysis of a skin biopsy revealed subepidermal blistering, while direct immunofluorescence showed the linear deposition of IgA and IgG antibodies at the dermoepidermal junction. Indirect immunofluorescence of normal human skin whose layers had been separated using 1M NaCl showed the binding of both IgA and IgG to the epidermal side. Immunoblotting demonstrated the presence of circulating IgA and IgG autoantibodies that bound to a 120-kDa protein. This is the first case of localized LAGBD whose skin lesions were restricted to the perianal region.
Department of Dermatology, Sapporo City General Hospital, Sapporo, Japan. satoko.shimizu@nifty.com
Satoko
S
Shimizu
Ken
K
Natsuga
Satoru
S
Shinkuma
Chikako
C
Yasui
Kikuo
K
Tsuchiya
Hiroshi
H
Shimizu
D000284:Administration, Oral; D000368:Aged; D001003:Anal Canal; D001323:Autoantibodies / Q000097:blood*; D001324:Autoantigens / Q000276:immunology*; D001706:Biopsy; D001768:Blister / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D003622:Dapsone / Q000008:administration & dosage; D019085:Fluorescent Antibody Technique, Direct; D019084:Fluorescent Antibody Technique, Indirect; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D015151:Immunoblotting; D007070:Immunoglobulin A / Q000097:blood*; D007074:Immunoglobulin G / Q000097:blood*; D008297:Male; D024041:Non-Fibrillar Collagens / Q000276:immunology*; D011239:Prednisolone / Q000008:administration & dosage; D012867:Skin / Q000187:drug effects / Q000276:immunology* / Q000473:pathology; D012872:Skin Diseases, Vesiculobullous / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D012883:Skin Ulcer / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D016896:Treatment Outcome; D000097805:Collagen Type XVII
D001323:Autoantibodies; D001324:Autoantigens; D003622:Dapsone; D005938:Glucocorticoids; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D024041:Non-Fibrillar Collagens; D011239:Prednisolone; D000097805:Collagen Type XVII