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病例报告:与家族性地中海热相关的严重脓皮病——3例患者对秋水仙碱治疗反应良好。

Case report: severe pyoderma associated with familial Mediterranean fever--favorable response to colchicine in three patients.

作者信息

Lugassy G, Ronnen M

机构信息

Institute of Hematology, Barzilai Medical Center, Ashkelon, Israel.

出版信息

Am J Med Sci. 1992 Jul;304(1):29-31. doi: 10.1097/00000441-199207000-00008.

Abstract

Familial Mediterranean fever (FMF) is an autosomal recessive disorder that occurs most frequently among Sephardic Jews and Armenians. It is characterized by recurrent episodes of fever, peritonitis, pleuritis, and arthritis. Skin lesions are seen in some patients. Diagnosis of FMF usually is made on clinical grounds only, typically when recurrent attacks of abdominal pain, fever, and arthritis are observed in a patient with an appropriate ethnic background and family history. To date, there are no specific diagnostic laboratory tests for FMF. Three patients with severe recurrent Pyoderma are covered in this report. In all three cases, the cutaneous lesions were associated with clinical manifestations of FMF and responded to colchicine therapy favorably. The importance of such an association and its therapeutic consequences are emphasized.

摘要

家族性地中海热(FMF)是一种常染色体隐性疾病,在西班牙裔犹太人和亚美尼亚人中最为常见。其特征为发热、腹膜炎、胸膜炎和关节炎反复发作。部分患者会出现皮肤病变。FMF通常仅基于临床症状进行诊断,典型情况是在具有适当种族背景和家族史的患者中观察到反复出现的腹痛、发热和关节炎发作。迄今为止,尚无针对FMF的特异性诊断实验室检查。本报告涵盖了3例严重复发性脓疱病患者。在所有3例病例中,皮肤病变均与FMF的临床表现相关,且对秋水仙碱治疗反应良好。强调了这种关联的重要性及其治疗后果。

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