Zhu Xuejun, Zhang Bingxin
Department of Dermatology, Peking University First Hospital, Beijing, China.
J Dermatol. 2007 Aug;34(8):503-11. doi: 10.1111/j.1346-8138.2007.00322.x.
Paraneoplastic pemphigus (PNP) is a life-threatening autoimmune blistering skin disease. Clinically, it is characterized by severe mucosal erosions and various cutaneous lesions associated with lymphoproliferative neoplasmas. Suprabasal acantholysis and clefts with scattered necrotic keratinocytes are the unique histopathological features. PNP patient sera recognize multiple antigens, which have been identified as the plakin protein family that includes desmoplakin, bullous pemphigoid antigen I (BPAG1), envoplakin and periplakin, and desmogleins 1 and 3. Castleman's tumor, non-Hodgkin's lymphoma, thymoma, follicular dendritic cell sarcoma and chronic lymphocytic leukemia are the commonly associated neoplasmas in PNP. We have also demonstrated that the autoantibodies reacting to epidermal proteins are directly produced by the cells in the associated tumors. Bronchiolitis obliterans is frequently found in PNP and may cause respiratory failure and death. In our experience, the early detection and removal of the tumor and i.v. administration of immunoglobulin are critical for the treatment of PNP.
副肿瘤性天疱疮（PNP）是一种危及生命的自身免疫性水疱性皮肤病。临床上，其特征为严重的黏膜糜烂以及与淋巴增殖性肿瘤相关的各种皮肤损害。基底层上棘层松解和伴有散在坏死角质形成细胞的裂隙是其独特的组织病理学特征。PNP患者血清可识别多种抗原，这些抗原已被确定为包括桥粒斑蛋白、大疱性类天疱疮抗原I（BPAG1）、内披蛋白和周皮蛋白的斑蛋白家族，以及桥粒芯糖蛋白1和3。卡斯特曼病、非霍奇金淋巴瘤、胸腺瘤、滤泡树突状细胞肉瘤和慢性淋巴细胞白血病是PNP中常见的相关肿瘤。我们还证明，与表皮蛋白反应的自身抗体是由相关肿瘤中的细胞直接产生的。闭塞性细支气管炎在PNP中经常出现，可能导致呼吸衰竭和死亡。根据我们的经验，早期发现并切除肿瘤以及静脉注射免疫球蛋白对PNP的治疗至关重要。
