Santi C G, Maruta C W, Aoki V, Sotto M N, Rivitti E A, Diaz L A
Department of Dermatology, University of São Paulo.
J Am Acad Dermatol. 1996 Jan;34(1):40-6. doi: 10.1016/s0190-9622(96)90832-4.
Pemphigus herpetiformis is a rare and atypical variant of pemphigus that resembles dermatitis herpetiformis. Most patients show antiepidermal autoantibodies that stain the epidermal intercellular spaces by immunofluorescence, similar to pemphigus autoantibodies, and lack the immunopathologic features of dermatitis herpetiformis.
The study was aimed at characterizing the specificity of the antiepidermal autoantibodies in seven patients with pemphigus herpetiformis.
The antiepidermal autoantibodies were characterized by immunofluorescence, immunoblotting, and immunoprecipitation studies in seven patients who fulfilled the clinical, histologic, and immunofluorescence diagnostic criteria for pemphigus herpetiformis.
Five patients with features of pemphigus herpetiformis either had classic pemphigus foliaceus, or their disease evolved into classic pemphigus foliaceus. One of these patients had fogo selvagem. Two of the seven patients showed features of or had disease that evolved into pemphigus vulgaris. The antiepidermal autoantibodies present in all seven patients recognized desmoglein 1.
Pemphigus herpetiformis is a rare clinical and histologic expression of nonendemic pemphigus foliaceus, fogo selvagem, and pemphigus vulgaris.
疱疹样天疱疮是一种罕见的、非典型的天疱疮变异型，类似于疱疹样皮炎。大多数患者表现出抗表皮自身抗体，通过免疫荧光法可使表皮细胞间间隙着色，类似于天疱疮自身抗体，且缺乏疱疹样皮炎的免疫病理特征。
本研究旨在明确7例疱疹样天疱疮患者抗表皮自身抗体的特异性。
通过免疫荧光、免疫印迹和免疫沉淀研究对7例符合疱疹样天疱疮临床、组织学和免疫荧光诊断标准的患者的抗表皮自身抗体进行特征分析。
5例具有疱疹样天疱疮特征的患者要么患有典型的落叶型天疱疮，要么其病情发展为典型的落叶型天疱疮。其中1例患者患有巴西落叶型天疱疮。7例患者中有2例表现出寻常型天疱疮的特征或病情发展为寻常型天疱疮。所有7例患者体内的抗表皮自身抗体均识别桥粒芯糖蛋白1。
疱疹样天疱疮是地方性落叶型天疱疮、巴西落叶型天疱疮和寻常型天疱疮罕见的临床和组织学表现。
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