Update: Dermatomyositis

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.

Abklärung und Differenzialdiagnose der Myositiden

“…Violette Papeln treten häufig über den Dorsalseiten der Metakarpophalangeal gelenke und den Interphalangealgelenken der Finger auf (Gottronsche Papeln). Hellrot sind die Erytheme vor allem bei der paraneoplastischen Dermatomyositis (5). Bei…”