Ng Patricia P L, Rencic Adrienne, Nousari H Carlos
Department of Dermatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
J Cutan Med Surg. 2002 Sep-Oct;6(5):434-7. doi: 10.1007/s10227-001-0130-x. Epub 2002 Aug 29.
We report on a 42-year-old man with Stage IIA non-Hodgkin's lymphoma who developed a severe mucocutaneous blistering eruption. His diagnosis, paraneoplastic pemphigus, was based on clinical, histological, and immunofluorescence findings and confirmed by immunoprecipitation. Despite maximal therapy with plasmapheresis, corticosteroids, and mycophenolate mofetil and the subsequent addition of cyclophosphamide and cyclosporine, the condition was fatal.
This case illustrates the refractory nature of this disease and the inadequacy of existing therapies.
我们报告一名42岁患有IIA期非霍奇金淋巴瘤的男性，他出现了严重的黏膜皮肤水疱性皮疹。他的诊断为副肿瘤性天疱疮，基于临床、组织学和免疫荧光检查结果，并通过免疫沉淀得以证实。尽管采用了血浆置换、皮质类固醇、霉酚酸酯进行最大程度的治疗，随后又加用了环磷酰胺和环孢素，但病情仍属致命。
本病例说明了该疾病的难治性以及现有治疗方法的不足。
