Wind Lynnda J N, van der Velden Annette W G, Diercks Gilles F H, Pas Hendri H, Jonkman Marcel F
Martini Ziekenhuis, Afd. interne geneeskunde, Groningen, The Netherlands.
Ned Tijdschr Geneeskd. 2010;154:A2183.
A 53-year-old man with non-Hodgkin lymphoma developed red, flaky skin, which was initially suggestive of a drug reaction. He also had pneumonia, for which he was admitted for antibiotic treatment. During admission the skin picture changed and blisters and erosions appeared on his body. Skin biopsy and immunological examination led to the diagnosis of paraneoplastic pemphigus (PNP). The patient died five months after the diagnosis of PNP due to PNP pneumonia. PNP is a rare and often aggressive bullous disease with an autoimmune pathogenesis, associated with underlying lymphoproliferative disease. It is characterised by a polymorphous skin rash, painful mucosal erosions, sometimes with respiratory complications due to bronchiolitis obliterans. Diagnosis is based on clinical, histological and immunological findings. The prognosis is unfavourable; death occurs in 90 percent of patients. This case illustrates the importance of histology, immunofluorescence microscopy, and immunoserology in misunderstood skin disorders in patients with lymphoproliferative disease.
一名53岁的非霍奇金淋巴瘤男性患者出现了皮肤发红、片状脱皮，最初怀疑是药物反应。他还患有肺炎，并因此入院接受抗生素治疗。住院期间，皮肤症状发生了变化，身上出现了水疱和糜烂。皮肤活检和免疫检查确诊为副肿瘤性天疱疮（PNP）。该患者在诊断为PNP五个月后因PNP肺炎死亡。PNP是一种罕见且通常具有侵袭性的大疱性疾病，其发病机制为自身免疫性，与潜在的淋巴增殖性疾病相关。其特征为多形性皮疹、疼痛性黏膜糜烂，有时还会因闭塞性细支气管炎出现呼吸系统并发症。诊断基于临床、组织学和免疫学检查结果。预后不佳，90%的患者会死亡。该病例说明了组织学、免疫荧光显微镜检查和免疫血清学在淋巴增殖性疾病患者被误解的皮肤疾病中的重要性。
