Paraneoplastic pemphigus with a pemphigus vegetans-like plaque as the only cutaneous manifestation
Paraneoplastic pemphigus with a pemphigus vegetans-like plaque as the only cutaneous manifestation
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DOI：
10.1016/s0190-9622(98)70368-8
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发表时间：
1998-11-01
影响因子：
13.8
通讯作者：
Anhalt, GJ
中科院分区：
文献类型：
--
作者：
Sapadin, AN;Anhalt, GJ
副肿瘤性天疱疮(PNP)是一种新近发现的自身免疫性黏膜皮肤病，与少数罕见的淋巴网状肿瘤有关。顽固性口腔炎是最常见的临床特征，但皮肤表现各不相同。我们描述一个与慢性淋巴细胞白血病相关的PNP患者，他表现为严重的口腔、喉部和咽部受累。唯一的皮肤发现是天疱疮素人样斑块，这是一种以前未描述的表现。斑块是从先前静脉导管部位形成的松弛水泡演变而来的。粘膜活检标本呈淡淡的细胞间免疫球蛋白染色，而皮肤斑块的结果为阴性。浴猴食道和大鼠膀胱上皮细胞间接免疫荧光试验均为阳性。免疫沉淀(IP)研究证实了4种蛋白质的复合体，它们的相对分子质量分别为250、230、210和190kd，证实了PNP的诊断。
Paraneoplastic pemphigus (PNP) is a recently recognized autoimmune mucocutaneous disease associated with a few unusual lymphoreticular neoplasms. Intractable stomatitis is the most constant clinical feature but the cutaneous presentations are characteristically variable. We describe a patient with PNP associated with chronic lymphocytic leukemia who presented with severe oral, laryngeal, and pharyngeal involvement. The only cutaneous finding was a pemphigus vegetans-like plaque, a previously undescribed manifestation. The plaque evolved from a flaccid blister that formed at a prior intravenous catheter site. The mucosal biopsy specimen demonstrated faint intercellular staining with IgG, whereas results for the cutaneous plaque were negative. Indirect immunofluorescence testing was positive on bath monkey esophagus and rat bladder epithelium. Immunoprecipitation (IP) studies demonstrated a complex of 4 proteins with molecular weights of 250, 230, 210, and 190 kd, confirming the diagnosis of PNP.