Histologic features of paraneoplastic pemphigus.
Histologic features of paraneoplastic pemphigus.
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DOI：
10.1001/archderm.1992.01680180085011
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发表时间：
1992-08
影响因子：
--
通讯作者：
T. Horn;G. Anhalt
中科院分区：
文献类型：
--
作者：
T. Horn;G. Anhalt
背景我们描述了副肿瘤性天疱疮的组织病理学特征，这是一种最近描述的与肿瘤相关的自身免疫性皮肤粘膜疾病。对副肿瘤性天疱疮的完整评估需要鉴定特征性皮肤粘膜出疹、用于常规组织学和直接免疫荧光评估的组织标本，以及鉴定具有独特特异性的循环自身抗体。角质形成细胞的免疫沉淀揭示了四种蛋白质与循环抗体的特征复合物。副肿瘤性天疱疮患者中已发现多种肿瘤。观察 我们回顾了来自 6 名副肿瘤性天疱疮患者的 16 份皮肤和口腔粘膜活检标本，经证实符合所有标准。主要特征包括表皮棘层松解、基底上裂形成、角化细胞角化不良、基底表皮空泡变化以及炎症细胞的表皮胞吐作用。 16 个样本中的 7 个（44%）显示整个表皮基底上棘层松解症和角化不良角质形成细胞的独特组合。这些组织学结果与被描述为具有天疱疮和多形红斑特征的特征性临床病变相对应。结论 副肿瘤性天疱疮是一种独特的临床、组织学和免疫学疾病，其特征是产生针对桥粒斑蛋白 I 和桥粒斑蛋白 II、大疱性类天疱疮抗原以及桥粒复合物中可能的其他抗原的自身抗体。对组织学特征的识别应促进对隐匿性肿瘤的免疫病理学确认和评估。
BACKGROUND We describe the histopathologic features of paraneoplastic pemphigus, a recently described autoimmune mucocutaneous disease associated with neoplasia. Complete evaluation for paraneoplastic pemphigus requires identification of the characteristic mucocutaneous eruption, tissue specimens for routine histologic and direct immunofluorescence evaluation, and identification of circulating autoantibodies with a unique specificity. Immunoprecipitation from keratinocytes reveals a characteristic complex of four proteins with the circulating antibodies. Various neoplasms have been identified in patients with paraneoplastic pemphigus. OBSERVATIONS We reviewed 16 skin and oral mucous membrane biopsy specimens from six patients with paraneoplastic pemphigus confirmed by fulfillment of all criteria. Major features include epidermal acantholysis, suprabasal cleft formation, dyskeratotic keratinocytes, vacuolar change of the basilar epidermis, and epidermal exocytosis of inflammatory cells. Seven (44%) of 16 specimens displayed a unique combination of suprabasal acantholysis and dyskeratotic keratinocytes throughout the epidermis. These histologic findings correspond to those of the characteristic clinical lesions that are described as having features of pemphigus and erythema multiforme. CONCLUSIONS Paraneoplastic pemphigus represents a unique clinical, histologic, and immunologic disease characterized by autoantibody production to desmoplakin I and desmoplakin II, bullous pemphigoid antigen, and, possibly, other antigens in the desmosomal complex. Recognition of the histologic features should prompt immunopathologic confirmation and evaluation for an occult neoplasm.