Calcinosis Cutis Occurring in Association With Autoimmune Connective Tissue Disease The Mayo Clinic Experience With 78 Patients, 1996-2009
Calcinosis Cutis Occurring in Association With Autoimmune Connective Tissue Disease The Mayo Clinic Experience With 78 Patients, 1996-2009
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DOI：
10.1001/archdermatol.2011.2052
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发表时间：
2012-04-01
影响因子：
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通讯作者：
Davis, Mark D. P.
中科院分区：
文献类型：
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作者：
Balin, Samuel J.;Wetter, David A.;Davis, Mark D. P.
目的：描述自身免疫性结缔组织病（ACTD）临床背景下皮肤钙质沉着症患者的特征和治疗。设计：回顾性研究。设置：三级转诊中心。患者：1996年至2009年间78例皮肤钙质沉着症和ACTD患者。主要结果指标：ACTD临床背景下皮肤钙质沉着症患者的临床特征、治疗和结局。结果：在78例患者中（皮肤钙质沉着症发病时的平均年龄为40.1岁），64例（82%）为女性。以下疾病与皮肤钙质沉着症相关：皮肌炎（n=30），经典型（n=15）、幼年型（n=14）和无肌病型（n=1）亚型;系统性硬化症伴局限性皮肤硬皮病（n=24）;狼疮性脂膜炎（n=4）;系统性红斑狼疮（n=2）;混合性结缔组织病（n=4）;重叠性结缔组织病（n=6）;未分化结缔组织病（n=6）;多发性肌炎（n=1）;和类风湿性关节炎（n=1）。皮肤钙质沉着症的治疗包括单独药物治疗（n=19）、单独手术治疗（n=11）、药物和手术联合治疗（n=17）、未治疗（n=30）和未知（n=1）。盐酸地尔硫卓是最常用的药物治疗方法，17名患者中有9名出现部分缓解。28例患者手术切除1个或多个病灶的皮肤钙质沉着症：22个有完全反应，5个有部分反应，1个没有respons.Conclusions：皮肌炎和系统性硬化症是最常见的ACTDs与皮肤钙质沉着症。虽然没有治疗是一致有效的，手术切除症状性病变和药物治疗与地尔硫卓提供了一些患者的好处。
Objective: To describe characteristics and treatment of patients with calcinosis cutis in the clinical setting of autoimmune connective tissue disease (ACTD).Design: Retrospective study.Setting: Tertiary referral center.Patients: Seventy-eight patients with calcinosis cutis and ACTD between 1996 and 2009.Main Outcome Measures: Clinical features, treatments, and outcomes of patients with calcinosis cutis in the clinical setting of ACTD.Results: Of 78 patients (mean age at onset of calcinosis cutis, 40.1 years), 64 (82%) were female. The following diseases were associated with calcinosis cutis: dermatomyositis (n=30) with classic (n=15), juvenile (n=14), and amyopathic (n=1) subtypes; systemic sclerosis with limited cutaneous scleroderma (n=24); lupus panniculitis (n=4); systemic lupus erythematosus (n=2); mixed connective tissue disease (n=4); overlap connective tissue disease (n=6); undifferentiated connective tissue disease (n=6); polymyositis (n=1); and rheumatoid arthritis (n=1). Therapy for calcinosis cutis consisted of medical treatment alone (n=19), surgical therapy alone (n=11), combined medical and surgical treatment (n=17), no treatment (n=30), and unknown (n=1). Diltiazem hydrochloride was the most commonly used medical therapy, with 9 of 17 patients having a partial response. Twenty-eight patients had surgical excision of 1 or more lesions of calcinosis cutis: 22 had a complete response, 5 had a partial response, and 1 had no response.Conclusions: Dermatomyositis and systemic sclerosis were the most common ACTDs associated with calcinosis cutis. Although no treatment was uniformly effective, surgical excision of symptomatic lesions and medical treatment with diltiazem provided benefit for some patients.