Hypomyopathic dermatomyositis

Last revised by Assoc Prof Craig Hacking on 18 Aug 2021

Hypomyopathic dermatomyositis is a rare systemic autoimmune disorder of the skin with little to no muscle involvement. It makes up <5% of all dermatomyositis diagnoses. 

The incidence is 2 in 1,000,000 ref and it typically affects women more than man at a 2:1 1.

Symptoms include fatigue, joint pain, fevers and/or dysphagia. Signs include periorbital violaceous plaques (heliotrope rash), Grotton papules (hand extensor surfaces),​ Raynaud phenomenon and/or a non-erosive inflammatory polyarthritis. Clinical muscle weakness is absent 1.

  • muscle/skin biopsy: dense lymphocytic infiltrate and atrophy of muscle fibers

With pulmonary involvement, CT can demonstrate diffuse ground-glass opacity 2 as well as features of pulmonary fibrosis (e.g. reticular opacity, honeycombing) ref.​

  • immunosuppressive therapy: tacrolimus, cyclosporin, prednisolone
  • anti-histamine medication: hydroxyzine
  • avoidance of sun exposure
  • cancer screening as appropriate to age, there is some debate as to whether increased screening would be warranted 3

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Cases and figures

  • Case 1: dermatomyositis-related ILD
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