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Acquired Epidermolysis Bullosa With the Clinical Feature of Brunsting-Perry Cicatricial Bullous Pemphigoid
Günter Kurzhals, MD;
Wilhelm Stolz, MD;
Michael Meurer, MD;
Johannes Kunze, MD;
Otto Braun-Falco, MD;
Thomas Krieg, MD
Arch Dermatol. 1991;127(3):391-395.
Abstract
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A 56-year-old woman with the typical clinical feature of cicatricial bullous pemphigoid of the Brunsting-Perry type was studied. Histologic examination of a lesional skin biopsy specimen demonstrated a subepidermal blister. Direct immunofluorescence microscopy revealed linear deposits of IgG, IgM, and C3 located on both the roof and the floor of the blister. Immunofluorescence antigen mapping using cryostat sections of a spontaneous blister and antisera against defined basement membrane components localized the bullous pemphigoid antigen and type IV collagen in the roof of the blister. This dermal type of blister formation was confirmed by electron microscopy, which showed the cleavage level below the lamina densa. In direct immunoelectron microscopy, granular deposits of C3 and IgG were found attached to and just beneath the lamina densa in a pattern identical to the distribution of anchoring fibrils. These findings are diagnostic of acquired epidermolysis bullosa, a blistering disease that has much more clinical heterogeneity than previously suggested.
(Arch Dermatol. 1991;127:391-395)
Author Affiliations
From the Departments of Dermatology, Ludwig-Maximilians, University of Munich (Federal Republic of Germany) (Drs Kurzhals, Stolz, Meurer, Braun-Falco, and Krieg) and St Barbara-Hospital Duisburg (Federal Republic of Germany) (Dr Kunze). Dr Kurzhals is now with the Department of Dermatology, Städtisches Krankenhaus München-Schwabing.
Footnotes
Accepted for publication October 23, 1990.
Reprint requests to Dermatologische Klinik und Poliklinik der Ludwig-Maximilians, Universität München, Frauenlobstr 9-11, D-8000 München 2, Federal Republic of Germany (Dr Krieg).
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FULL TEXT
Localized Cicatricial Pemphigoid of the Brunsting-Perry Type With Transition Into Disseminated Cicatricial Pemphigoid: Report of a Case Proved by Preembedding Immunogold Electron Microscopy
Kurzhals et al.
Arch Dermatol 1995;131:580-585.
ABSTRACT
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