中华皮肤科杂志 ›› 2013, Vol. 46 ›› Issue (9): 669-670.

• 研究报道 • 上一篇    下一篇

硬肿病八例临床分析

吴萍1,吴金琼2,王立3,吴迪3,陈华3,刘跃华4,方凯5,何春霞6,侯勇3,曾小峰3,张奉春3   

  1. 1. 河北省唐山市工人医院风湿免疫科
    2. 广西壮族自治区桂平市人民医院肾内风湿科
    3. 北京协和医院风湿免疫科
    4. 北京协和医院皮肤科
    5.
    6. 中国医学科学院北京协和医学院北京协和医院
  • 收稿日期:2013-01-22 修回日期:2013-05-15 出版日期:2013-09-15 发布日期:2013-09-01
  • 通讯作者: 王立 E-mail:wangli2221@sina.com

Clinical analysis of eight cases of scleredema

  • Received:2013-01-22 Revised:2013-05-15 Online:2013-09-15 Published:2013-09-01

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摘要： 【摘要】 目的 探讨硬肿病的临床特点。方法 北京协和医院1998—2012年住院治疗的8例硬肿病患者的临床表现、病理特征及治疗转归。 结果 男女患者各4例，平均年龄44岁。皮肤变硬增厚累及颈肩、背部6例；合并多系统受累6例；2型糖尿病5例中3例有糖尿病并发症；2例出现血清异常免疫球蛋白，λ、κ型M蛋白各1例；发热2例；合并干燥综合征1例。4例行皮肤活检，组织病理主要表现为真皮全层增厚，胶原增生明显，胶原纤维空隙可见阿新蓝染色阳性。8例中3例接受糖皮质激素、免疫抑制剂治疗有效，1例青霉素治疗有效，1例紫外线治疗效果欠佳。结论 硬肿病患者应常规监测血糖、筛查血清免疫球蛋白，警惕脏器受累及多发性骨髓瘤、淋巴瘤等恶性疾病。

关键词: 硬肿病

Abstract: WU Ping，WU Jin-qiong，WANG Li *，WU Di，CHEN Hua，LIU Yue-hua，FANG Kai, HE Chun-xia, HOU Yong, ZENG Xiao-feng， ZHANG Feng-chun. *Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College; Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China Corresponding author: WANG Li, Email: wangli2221@sina.com 【Abstract】 Objective To assess the clinical characteristics of patients with scleredema. Methods This retrospective study included eight patients with scleredema hospitalized in the Peking Union Medical College Hospital（PUMCH） from 1998 to 2012. Data were collected from medical records. Clinical manifestations, pathological findings and therapeutic outcomes were analyzed. Results There were four male patients and four female patients with an average age of 44 years. Sclerotic induration of the skin on the neck, shoulders and back were observed in 6 （75%） patients, and multi-systemic involvement in 6 patients. Three of five patients with type 2 diabetes millitus（DM） suffered from diabetes-associated complications. Lambda type M-protein was observed in one patient, and Kappa type M-protein in one patient. Two patients also suffered from fever, and one from Sj?觟gren′s syndrome. Pathological examination of biopsy specimens from four patients revealed thickening of the whole dermis as well as marked hyperpasia of collagen. Alcian blue staining was positive in the space between collagen fibers. The condition was improved in three patients treated with glucocorticoid and immunosuppressants as well as in one patient receiving benzylpenicillin treatment, but poorly responded to ultraviolet therapy in one patient. Conclusions It is recommended to monitor blood glucose and serum immunoglobulins as well as to screen for the involvement of multiple systems and occurrence of multiple myeloma, lymphoma and other malignancies among patients with scleredema.