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Herpetiform pemphigus showing reactivity with pemphigus vulgaris antigen (desmoglein 3)
Authors:A. KUBO&dagger  ,M. AMAGAL§  ,T. HASHIMOTO&Dagger  ,T. DOI,M. HIGASHIYAMA,K. HASHIMOTO¶  ,K. YOSHIKAWA
Affiliation:Department of Dermatology, Osaka University School of Medicine, Japan;Department of Cell Biology, Kyoto University Faculty of Medicine, Japan;Department of Dermatology, Keio University School of Medicine, Japan;Department of Dermatology, Kurwne University School of Medicine, Japan;Department of Dermatology, Ehime University School of Medicine, Japan
Abstract:We report a patient with herpetiform pemphigus(HP)who showed reactivity only with pemphigus vulgaris(PV)antigen but not with pemphigus foliaceus(PF)antigen. Direct and indirect immuno- fluorescence revealed keratinocyte cell surface staining in the lower layers of the epidermis, where desmoglein 3 (Dsg3) is expressed. Immunoblot analysis, using ethylenediamine tetra-acetic acid- separated human epidermal extracts, revealed that the patient's serum recognized only a 130-kDa polypeptide which co-migrated with Dsg3. By antigen-specific immunoadsorption studies, using desmoglein 1 (Dsg1) and Dsg3 recombinant protein produced by baculovirus expression system, immunoreactivity of the patient's serum was completely adsorbed by Dsg3 alone, but not by Dsg1. These results indicate that this HP patient produced only anti-Dsg3 autoantibodies and no other autoantibodies against components of the keratinocyte cell surface. HP could be a variant of PV.in addition to PF, with unique clinical and histological features.
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