临床无肌病性皮肌炎

Clinically Amyopathic Dermatomyositis

临床无肌病性皮肌炎(C-ADM)是指一组仅有皮肤表现或以皮肤表现为主、不伴有近端肌无力和血肌酶谱升高的皮肌炎(DM),包括无肌病性皮肌炎和微肌病性皮肌炎。该病在欧美的发病率约占 DM 的10%.在亚洲这一比例可能要高些。C-ADM 的皮肤表现与经典 DM 一致.随着病程的进展,大部分 C-ADM 患者并不会出现肌炎的表现。但是,C-ADM 常合并有可能危及生命的间质性肺病和恶性肿瘤,故早期诊断、治疗十分重要,特异性自身抗体的检测将有助于该病的早期诊断。治疗方法目前尚无成熟统一的方案,主要针对皮肤局部病变和严重的合并症进行治疗。

Clinically amyopathic dermatomyositis （C-ADM）, including amyopathic dermatomyositis （DM） and hypomyopathic DM, is a subset of DM characterized by skin-only or skin-predominant manifestations with no clinical evidence of proximal muscle weakness and no serum muscle enzyme elevation. C-ADM accounts for approximately 10% of classical DM in Caucasians and possibly a higher incidence in Asians. The cutaneous manifestations of C-ADM are clinically and histopathologically identical to those of classical DM. Most patients with C-ADM will not progress to myopathy for a long time. However, C-ADM is often associated with potentially life-threatening interstitial lung disease and malignancy, so it is important to diagnose and treat C-ADM as early as possible. Specific autoantibodies can help for early diagnosis of C-ADM. So far, there is no standard regimen for the management of C-ADM and the main aim of the treatment is to control the topical skin lesions and severe systemic manifestations.