Autoimmune Skin Disease Program, Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, 221 Longwood Avenue, Boston, MA, 02115, USA.
Ronald O. Perelman Department of Dermatology, New York University Langone Medical Center, New York University School of Medicine, New York, NY, USA.
Am J Clin Dermatol. 2020 Jun;21(3):339-353. doi: 10.1007/s40257-020-00502-6.
Dermatomyositis is a rare inflammatory disease with characteristic cutaneous findings and varying amounts of systemic involvement. Patients may present with skin disease alone, have concomitant muscle disease, or have extracutaneous manifestations such as pulmonary disease or an associated malignancy. Given such diverse presentations, dermatomyositis is both a diagnostic and therapeutic challenge. However, a prompt diagnosis is of utmost importance to institute adequate therapy and screen patients for an associated malignancy. Dermatologists should play a crucial role in the diagnosis and management of patients with dermatomyositis as cutaneous disease tends to be chronic, negatively impact quality of life, and be more recalcitrant to therapy. In this review, we discuss diagnosis, with a focus on myositis-specific antibodies and their associated phenotypes. We also review therapies available for this often refractory skin disease.
皮肌炎是一种罕见的炎症性疾病，具有特征性的皮肤表现和不同程度的全身受累。患者可能仅表现为皮肤疾病，也可能同时存在肌肉疾病，或有肺部疾病或相关恶性肿瘤等皮肤外表现。鉴于如此多样的表现，皮肌炎既是诊断的挑战，也是治疗的挑战。然而，及时诊断对于实施适当的治疗和筛查相关恶性肿瘤至关重要。皮肤科医生应在皮肌炎患者的诊断和管理中发挥关键作用，因为皮肤疾病往往是慢性的，会对生活质量产生负面影响，并且对治疗更具抗性。在这篇综述中，我们讨论了诊断，重点是肌炎特异性抗体及其相关表型。我们还回顾了这种常见的难治性皮肤病的可用疗法。
