Wick M R, Thomas J R, Scheithauer B W, Jackson I T
Arch Dermatol. 1983 May;119(5):409-14.
Multifocal Merkel's cell tumors developed in a 24-year-old patient with an unusual cutaneous dysplasia syndrome. The neoplasms behaved in an aggressive fashion, with the development of multiple lymph node metastases. In this case, the dermatologic syndrome had features of both dyshidrotic ectodermal dysplasia and the basal cell nevus syndrome. In addition to Merkel's cell tumors, basal cell carcinomas and actinic elastosis were seen in sun-exposed skin sites. These findings, in conjunction with a known tendency for Merkel's cell tumors to arise in solar-damaged areas, suggest a pathogenetic relationship between these neoplasms and the underlying cutaneous syndrome in this patient.
一名患有罕见皮肤发育异常综合征的24岁患者发生了多灶性默克尔细胞肿瘤。这些肿瘤表现出侵袭性，出现了多处淋巴结转移。在该病例中，皮肤病综合征具有汗疱疹性外胚层发育不良和基底细胞痣综合征的特征。除了默克尔细胞肿瘤外，在阳光暴露的皮肤部位还可见基底细胞癌和光化性弹性组织变性。这些发现，结合默克尔细胞肿瘤在日光损伤区域发生的已知倾向，提示了这些肿瘤与该患者潜在皮肤综合征之间的发病机制联系。
