A Case of Nonscarring Subepidermal Blistering Disease Associated with Autoantibodies Reactive with Both Type VII Collagen and Laminin 5

Abstract

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 α₃- and β₃-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.

IgG anti-laminin-332 autoantibodies are present in a subset of patients with mucous membrane, but not bullous, pemphigoid

“…While bullous disease patients with autoantibodies against multiple autoantigens (including L-332) in epidermal BM have been described, such patients are relatively rare [25][26][27][28][29][30] . In contrast, a recent study found that 29 of 72 patients with BP (40%) had circulating IgG reactive with L-332 by ELISA 9 .…”

A Case of Epidermolysis Bullosa Acquisita with Autoantibody to Anti-p200 Pemphigoid Antigen and Exfoliative Esophagitis

“…The coexistence of autoantibodies to type VII collagen and laminin 5 or BP antigen has been reported [2,3,4,5]. On the other hand, anti-p200 pemphigoid complicated by another subepidermal autoimmune bullous disease is very rare.…”

“…The cases of subepidermal blistering disease associated with more than two antigens have rarely been reported [2,3,4,5]. We describe a case of a subepidermal blistering disease associated with autoantibodies to both type VII collagen and anti-p200 pemphigoid antigen (p200) which was accompanied with exfoliative esophagitis.…”

A Case of Mixed Bullous Disease of Epidermolysis bullosa acquisita and Linear IgA Bullous Dermatosis

“…Several similar cases, however, have been previously reported. We have reported a nonscarring subepidermal blistering disease associated with autoantibodies reactive with both type VII collagen and laminin 5 that could be diagnosed as coexistence of EBA and cicatricial pemphigoid [3]. Jonkman et al [2] described a case of EBA with IgG autoantibodies against type VII collagen and laminin a 3 .…”

“…It has been reported that certain autoimmune bullous diseases may have double distinct autoantibodies against the skin in the same patient [1,2,3,4,5]. However, the pathophysiological significances of two distinct autoantibodies against the skin in an autoimmune bullous dermatosis are not fully understood.…”