[Update: dermatomyositis].
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.
Volc-Platzer
B
B
Fachärztin für Haut- und Geschlechtskrankheiten, Wien, Osterreich, beatrix.volc-platzer@wienkav.at.
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D003882:Dermatomyositis / Q000175:diagnosis* / Q000188:drug therapy*; D006801:Humans; D007136:Immunoglobulins / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*
D000305:Adrenal Cortex Hormones; D007136:Immunoglobulins; D007166:Immunosuppressive Agents