Sontheimer Richard D
Department of Dermatology, University of Iowa College of Medicine/University of Iowa Hospitals & Clinics (UI Health Care), 200 Hawkins Drive, BT2045-1, Iowa City, IA 52242-1090, USA.
Dermatol Clin. 2002 Jul;20(3):387-408. doi: 10.1016/s0733-8635(02)00021-9.
Important points regarding DM and C-ADM are as follows: C-ADM is a working functional designation for patients having the skin-only and skin-predominant subsets of DM, amyopathic DM, and hypomyopathic DM. C-ADM seems to have approximately 10% the incidence of classic DM in whites and possibly a higher incidence in Asians. Some patients who present with C-ADM, with or without subclinical laboratory abnormalities, can slowly progress to develop symptomatic muscle weakness over a period of years, whereas others go for 10 to 20 years and longer without the appearance of muscle weakness. C-ADM patients are at risk for potentially life-threatening complications of classic DM, such as interstitial lung disease, which may occur in up to 10% of C-ADM patients. This risk seems to be even greater in some ethnic subgroups (e.g., Japanese). C-ADM patients may also be at increased risk for internal malignancy and until further studies are carried out to confirm the statistical significance of this association, all such patients should have a thorough evaluation for internal malignancy, identical to the approach currently used in classic DM patients. Dermatologists are in the best position initially to diagnose C-ADM patients and can contribute greatly to their overall management and quality of life. Ongoing vigilance is required, however, for complications that can arise in C-ADM patients including potentially fatal interstitial lung disease, internal malignancy, delayed onset of muscle weakness from myositis, and complications of systemic drug therapy. Topical therapy with broad-spectrum sunscreens, anti-inflammatories, and antipruritics should be maximized during the initial management of the cutaneous manifestations of either classic DM or C-ADM. Single-agent or combined aminoquinoline antimalarial therapy represents the safest initial form of systemic therapy for DM-specific skin disease occurring in any clinical setting; however, this approach tends to be less effective in general than for cutaneous LE. There is a theoretical rationale for and limited preliminary successful anecdotal experience with the use of anti-TNF-alpha therapy in refractory cases of classic DM and C-ADM. Cautious systematic clinical trials in this area should be considered.
关于局限性皮肌炎（C-ADM）和皮肌炎（DM）的要点如下：C-ADM是用于患有仅累及皮肤型、以皮肤为主型皮肌炎、无肌病性皮肌炎和低肌病性皮肌炎患者的一种实用功能性命名。在白种人中，C-ADM的发病率似乎约为经典DM的10%，在亚洲人中发病率可能更高。一些表现为C-ADM的患者，无论有无亚临床实验室异常，在数年时间里可能会缓慢进展为出现有症状的肌肉无力，而其他患者10至20年甚至更长时间都不会出现肌肉无力。C-ADM患者有发生经典DM潜在危及生命并发症的风险，如间质性肺病，在高达10%的C-ADM患者中可能发生。在某些种族亚组（如日本人）中，这种风险似乎更大。C-ADM患者发生内部恶性肿瘤的风险也可能增加，在进一步研究证实这种关联的统计学意义之前，所有此类患者都应像目前对经典DM患者采用的方法一样，进行全面的内部恶性肿瘤评估。皮肤科医生最初最适合诊断C-ADM患者，并且可以对其整体管理和生活质量做出很大贡献。然而，对于C-ADM患者可能出现的并发症需要持续警惕，包括潜在致命的间质性肺病、内部恶性肿瘤、肌炎导致的肌肉无力延迟发作以及全身药物治疗的并发症。在经典DM或C-ADM皮肤表现的初始管理期间，应最大限度地使用广谱防晒霜、抗炎药和止痒药进行局部治疗。单药或联合氨基喹啉抗疟药治疗是任何临床情况下发生的DM特异性皮肤病最安全的初始全身治疗形式；然而，一般来说，这种方法的效果往往不如用于皮肤型红斑狼疮。在经典DM和C-ADM的难治性病例中使用抗TNF-α治疗有理论依据且有有限的初步成功经验。应考虑在该领域进行谨慎的系统临床试验。
