A case of nonscarring subepidermal blistering disease associated with autoantibodies reactive with both type VII collagen and laminin 5.
A 35-year-old Japanese woman had recurrent, pruritic, vesicular lesions on the face, neck and upper back as well as erosive lesions of the oral cavity and genitalia. The skin and mucosal lesions healed without scarring upon the systemic administration of corticosteroid and azathioprine. Direct immunofluorescence revealed linear deposits of IgG, IgA and C3 at the cutaneous basement membrane zone. Indirect immunofluorescence on 1 M NaCl-split human skin sections demonstrated that the patient's IgG antibodies reacted with the dermal side of the split, while IgA antibodies weakly reacted with the epidermal side. By immunoblot analyses, the patient's serum reacted with the NC1 domain of type VII collagen as well as both the alpha3- and beta3-subunits of laminin 5. We regarded our case as a nonscarring subepidermal blistering disease with autoantibodies to both type VII collagen and two different subunits of laminin 5. Such a case has not been previously reported.
Umemoto
Naoka
N
Department of Dermatology, Jichi Medical School - Omiya Medical Center, Omiya, Saitama, Japan.
Demitsu
Toshio
T
Toda
Sunao
S
Ohsawa
Masumi
M
Noguchi
Tadahide
T
Kakurai
Maki
M
Yamada
Tomoko
T
Suzuki
Masayuki
M
Nakagawa
Hidemi
H
Komai
Ayako
A
Hashimoto
Takashi
T
D000328:Adult; D001323:Autoantibodies / Q000276:immunology*; D001379:Azathioprine / Q000008:administration & dosage; D001623:Betamethasone / Q000008:administration & dosage; D001707:Biopsy, Needle; D024143:Collagen Type VII / Q000276:immunology*; D004359:Drug Therapy, Combination; D016107:Epidermolysis Bullosa Acquisita / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology*; D005260:Female; D019085:Fluorescent Antibody Technique, Direct; D005500:Follow-Up Studies; D006801:Humans; D015151:Immunoblotting; D007150:Immunohistochemistry; D007797:Laminin / Q000276:immunology*; D011239:Prednisolone / Q000008:administration & dosage; D012008:Recurrence; D018570:Risk Assessment; D012720:Severity of Illness Index; D016896:Treatment Outcome
D001323:Autoantibodies; D024143:Collagen Type VII; D007797:Laminin; D001623:Betamethasone; D011239:Prednisolone; D001379:Azathioprine