A Case of Unique Subepidermal Blistering Disease with Autoantibodies against a Novel Dermal 200-kD Antigen

Abstract

Background: Several autoimmune subepidermal blistering diseases with autoantibodies against the epidermal basement membrane zone (BMZ) have been identified. Each shows distinct immunological findings. Objectives: Our patient showed clinical features which were indicative of bullous pemphigoid or linear IgA bullous dermatosis. In his serum, circulating IgG antibodies binding to the dermal side of skin split with 1 M NaCl were detected. To clarify the immunological character of the patient, we performed further studies. Methods: Western immunoblot analysis using normal human skin extracts and indirect immunofluorescence (IF) with affinity-purified IgG antibodies to the detected dermal protein were done. Results: Western immunoblot analysis demonstrated IgG antibodies reacting with a dermal 200-kD protein. Affinity-purified IgG antibodies to the 200-kD protein reacted to the dermal side of the split by indirect IF. Therefore, this dermal 200-kD protein is believed to be the epidermal BMZ antigen. Conclusion: Our patient showed an immunologically unique subepidermal blistering disease.

Anti‐p200 pemphigoid: A novel autoimmune subepidermal blistering disease

“…Antibodies from patients that recognize the 200‐kDa bands in immunoblot analysis, were eluted from nitrocellulose and subsequently examined by indirect immunofluorescence microscopy on NaCl‐split skin. They reacted with the dermal side of the split which confirms that it is the antibodies to p200 that actually cause the dermal staining signal 11,22 . Three patients with anti‐p200 pemphigoid showed a weak additional reactivity with BP230 11,22 .…”

“…Lesions usually heal without scarring; milia formation has been observed in some cases 19,20 . Oral and/or genital mucous membranes are affected in approximately 20% of patients with anti‐p200 pemphigoid 12,13,22,23 . Recently, an unusual case of anti‐p200 pemphigoid with predominant involvement of mucous membranes was reported 23 .…”

“… 12–16,19–21,24,25 Occasionally, formation of microabscesses at the tips of dermal papillae may be observed 13,14,19,21,24 . In a minority of patients, various numbers of eosinophilic granulocytes have been reported within the inflammatory infiltrate, resulting in a microscopic picture reminiscent of BP 11,22,23 …”

“…The course of the anti‐p200 pemphigoid is variable. While some patients remain in remission after tapering of immunosuppressive medication, 12,15 others experience repeated relapses requiring maintenance treatment over months and years 16,19,21–25 …”

A Case of Anti-p200 Pemphigoid with Autoantibodies against both a Novel 200-kD Dermal Antigen and the 290-kD Epidermolysis bullosa acquisita Antigen

“…Anti-p200 pemphigoid is a recently described autoimmune bullous disease with autoantibodies against a 200-kD dermal antigen [1, 2, 3, 4, 5, 6, 7, 8, 9, 10]. Histologically, these cases show subepidermal blisters with neutrophil and eosinophil infiltration, and by indirect immunofluorescence (IF) there are circulating IgG autoantibodies against the dermal side of skin split with NaCl.…”