Caproni Marzia, Cardinali Carla, Parodi Aurora, Giomi Barbara, Papini Manuela, Vaccaro Mario, Marzano Angelo, De Simone Clara, Fazio Marcello, Rebora Alfredo, Fabbri Paolo
Dipartimento di Scienze Dermatologiche, Clinica Dermatlogica II, Università degli Studi di Firenze, Firenze, Italy.
Arch Dermatol. 2002 Jan;138(1):23-7. doi: 10.1001/archderm.138.1.23.
To analyze the average age, sex distribution, duration of follow-up, clinical course, serologic abnormalities, and incidence of possibly associated malignancy in patients with amyopathic dermatomyositis.
Retrospective study.
University hospitals.
Thirteen patients with amyopathic dermatomyositis.
The 13 patients represented 8.2% of 157 patients with dermatomyositis seen retrospectively in a 10-year period by the Italian Group of Immunodermatology of the Italian Society of Dermatology and Venereology. Gottron papules and sign and periungual telangiectasias were found in approximately 50% of cases (papules in 7 patients, Gottron sign and periungual telangiectasias in 6), while periorbital violaceous erythema was seen in 70% (9 patients). Arthralgias occurred in 2 patients and Raynaud phenomenon in 4. An elevated erythrocyte sedimentation rate was detected in 6 patients, hepatitis B virus antigen in 3, speckled antinuclear antibodies in 7, and anti-Ro and antimitochondrial antibodies in 1 case each. None of our patients had evidence of internal malignancy. Neither cardiopulmonary nor esophageal dysfunction was demonstrated. Electromyography showed a protopathic muscle abnormality in 3 patients. Muscle biopsy disclosed myositis and a neurogenic myopathy in another one.
Amyopathic dermatomyositis is a rare disease. So far, only 2 series of a few cases each have been reported. The "amyopathic" subset of dermatomyositis is peculiar in that its cutaneous lesions are predominant for long periods or even permanently, although they are indistinguishable from those of classic dermatomyositis. The minimal or absent muscle disease and the rarity of serum immunologic findings imply a favorable prognosis in white patients.
分析无肌病性皮肌炎患者的平均年龄、性别分布、随访时间、临床病程、血清学异常以及可能相关恶性肿瘤的发生率。
回顾性研究。
大学医院。
13例无肌病性皮肌炎患者。
在意大利皮肤性病学会免疫皮肤病学意大利小组对157例皮肌炎患者进行的为期10年的回顾性研究中，这13例患者占8.2%。约50%的病例出现Gottron丘疹、征和甲周毛细血管扩张（7例有丘疹，6例有Gottron征和甲周毛细血管扩张），而70%（9例）出现眶周紫红色红斑。2例患者有关节痛，4例有雷诺现象。6例患者红细胞沉降率升高，3例乙肝病毒抗原阳性，7例斑点型抗核抗体阳性，各有1例抗Ro抗体和抗线粒体抗体阳性。我们的患者均无内部恶性肿瘤的证据。未发现心肺或食管功能障碍。3例患者肌电图显示原发性肌肉异常。另一例肌肉活检显示有肌炎和神经源性肌病。
无肌病性皮肌炎是一种罕见疾病。迄今为止，仅报道过2组各有少数病例的系列研究。皮肌炎的“无肌病”亚型很特殊，因为其皮肤损害在很长一段时间甚至永久占主导地位，尽管与经典皮肌炎的皮肤损害无法区分。肌肉疾病轻微或无，血清免疫学检查结果罕见，提示白种患者预后良好。
