Hsiao Pa-Fan, Hsiao Cheng-Hsiang, Tsai Tsen-Fang, Yao Ming, Jee Shiou-Hwa
Department of Dermatology, Mackay Memorial Hospital, Taipei, Taiwan.
Am J Clin Dermatol. 2009;10(1):29-32. doi: 10.2165/0128071-200910010-00004.
We describe a case of granulomatous slack skin in a 31-year-old woman with an unusual presentation of acquired ichthyosis and muscular masses involving four limbs over 3 years. Vesicles and ulcerative skin nodules first appeared only 3 months prior to diagnosis. The diagnosis was confirmed after sequential biopsies of muscle, skin lesions, and lymph nodes, together with molecular genetic studies. The patient responded poorly to various therapies, including thalidomide, and died of doxorubicin-related cardiomyopathy.
我们描述了一例31岁女性的肉芽肿性皮肤松弛症,该患者有获得性鱼鳞病的异常表现,且四肢出现肌肉肿块长达3年。水疱和溃疡性皮肤结节在诊断前3个月才首次出现。通过对肌肉、皮肤病变和淋巴结进行系列活检,并结合分子遗传学研究,确诊了该病例。患者对包括沙利度胺在内的各种治疗反应不佳,最终死于阿霉素相关性心肌病。