Update: Dermatomyositis

作者：Volc Platzer B^*

来源：Hautarzt, 2010, 61(1): 69-78.

DOI：10.1007/s00105-009-1897-8

• Inflammatory idiopathic myopathy
• Heliotrope erythema
• Gottron papules
• Muscle-specific antibodies
• Paraneoplasia

Dermatomyositis is an idiopathic inflammatory myopathy and an "orphan disease" (incidence 1:100,000). It comprises a heterogenous clinical spectrum with periorbital heliotrope erythema, acral Gottron papules, and proximal muscle weakness. Muscle-specific antibody profiles correlate with clinical variants. Overlap with other collagen vascular disorders occurs and about one-third of patients have an underlying malignancy (paraneoplastic dermatomyositis). High-dose oral corticosteroids are the mainstay of treatment, given until improvement of muscle symptoms and/or normalization of muscle enzymes Additional options include steroid-sparing immunosuppressants, or high dose intravenous immunoglobulins. Prognosis has improved considerably since use of high-dose corticosteroids, with about 90% of patients responding. Follow-up and search for a possible malignancy should be performed yearly.

• 出版日期2010-1
• 单位河南工业大学