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  • Title: [Update: dermatomyositis].
    Author: Volc-Platzer B.
    Journal: Hautarzt; 2010 Jan; 61(1):69-78; quiz 79. PubMed ID: 20091390.
    Abstract:
    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.
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