Paraneoplastic pemphigus
Paraneoplastic pemphigus
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DOI：
10.1111/j.1440-0960.2012.00921.x
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发表时间：
2013-11
影响因子：
2
通讯作者：
A. Yong;H. Tey
中科院分区：
文献类型：
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作者：
A. Yong;H. Tey
副肿瘤性天疱疮（PNP）是一种独特的自身免疫性起泡疾病，可影响皮肤以外的多个器官。它与某些肿瘤有关，其中最常与淋巴增生性疾病有关。PNP的临床表现通常是疼痛，严重的口腔糜烂，可能伴有全身皮肤疹和全身受累。皮疹可能有不同的形态，包括类似天疱疮、类天疱疮、多形性红斑或移植物抗宿主病的病变，以及类似扁平苔藓的病变。同样，组织学结果也显示出相当大的变异性。PNP的特点是存在针对各种抗原的自身抗体：desmoplakin I (250 kd)，大疱性类天疱疮抗原I (230 kd), desmoplakin II (210 kd), envoplakin (210 kd), periplakin (190 kd), plectin （500 kd）和170‐kd蛋白。这种170 - kd的蛋白最近被鉴定为α - 2 -巨球蛋白样蛋白- 1，是一种广泛的蛋白酶抑制剂，在分层上皮和其他PNP损伤组织中表达。PNP的预后较差，往往是致命的。通常需要免疫抑制剂来减少水泡，并且用化疗治疗潜在的恶性肿瘤可以控制自身抗体的产生。当PNP与良性肿瘤相关时，预后较好，这些肿瘤应尽可能手术切除。
Paraneoplastic pemphigus (PNP) is a distinct autoimmune blistering disease that can affect multiple organs other than the skin. It occurs in association with certain neoplasms, among which lymphoproliferative diseases are most commonly associated. The clinical presentation of PNP consists typically of painful, severe oral erosions that may be accompanied by a generalised cutaneous eruption and systemic involvement. The eruption may be of different morphology, consisting of lesions that resemble pemphigus, pemphigoid, erythema multiforme or graft versus host disease, as well as lesions resembling lichen planus. Similarly, the histological findings also show considerable variability. PNP is characterised by the presence of autoantibodies against various antigens: desmoplakin I (250 kd), bullous pemphigoid antigen I (230 kd), desmoplakin II (210 kd), envoplakin (210 kd), periplakin (190 kd), plectin (500 kd) and a 170‐kd protein. This 170‐kd protein has recently been identified as alpha‐2‐macroglobulin‐like‐1, a broad range protease inhibitor expressed in stratified epithelia and other tissue damaged in PNP. The prognosis of PNP is poor and the disease is often fatal. Immunosuppressive agents are often required to decrease blistering, and treating the underlying malignancy with chemotherapy may control autoantibody production. The prognosis is better when PNP is associated with benign tumours and these should be surgically excised when possible.