Servicio de Dermatología, Hospital Universitario Virgen de las Nieves, Granada, Spain.
Departamento de Dermatología, Facultad de Medicina, Universidad de Granada, Granada, Spain.
Dermatol Ther. 2021 May;34(3):e14942. doi: 10.1111/dth.14942. Epub 2021 Mar 23.
The term clinically amyopathic dermatomyositis (CADM) is used to represent a subgroup of patients with the typical cutaneous features of dermatomyositis (DM) in the absence of muscle involvement. Similar to classic DM, CADM can be associated with other connective tissue disorders and systemic manifestations such as interstitial lung disease and malignancy. Owing to the frequent discordance between muscle response and skin disease, the therapeutic approach to CADM represents a challenge. The current literature suggests that CADM treatment should follow a specific protocol, influenced by visceral involvement and the expression of certain myositis-specific antibodies, and different from the recommendation in the presence of myositis. Here, we present five new cases of CADM. We describe the available therapeutic options for skin manifestations in this type of DM, and we propose a step-by-step therapeutic scheme, using the cutaneous dermatomyositis disease area and severity index to assess response. Our literature review establishes mycophenolate mofetil and intravenous immunoglobulin as the most frequently successful therapies in refractory skin disease.
临床上无肌病性皮肌炎（CADM）这一术语用于代表一组具有皮肌炎（DM）典型皮肤表现但无肌肉受累的患者亚群。与经典 DM 相似，CADM 可与其他结缔组织疾病和全身表现相关，如间质性肺病和恶性肿瘤。由于肌肉反应和皮肤疾病之间经常存在不一致，CADM 的治疗方法具有挑战性。目前的文献表明，CADM 的治疗应遵循特定的方案，该方案受内脏受累和某些肌炎特异性抗体表达的影响，与存在肌炎时的建议不同。在这里，我们介绍了 5 例新的 CADM 病例。我们描述了这种类型的 DM 中皮肤表现的可用治疗选择，并提出了一个分步治疗方案，使用皮肤皮肌炎疾病面积和严重程度指数来评估反应。我们的文献复习确立了霉酚酸酯和静脉注射免疫球蛋白是治疗难治性皮肤疾病最常成功的治疗方法。
