Citation, DOI and article data
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
- autoimmune panel: positive antinuclear antibodies (ANA) and anti-MDA5 antibody
- creatine kinase: elevated
Treatment and prognosis
- 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
- 1. Mijares MC, Aldahan AS, Gonzalez HH, Benhayoun N, Alboukrek D. Hypomyopathic Dermatomyositis Presenting with Idiopathic CD4 T-lymphocytopenia and Delayed Anti-MDA5 Positivity. (2019) Cureus. 11 (2): e4133. doi:10.7759/cureus.4133 - Pubmed
- 2. Makiko Yashiro et al. AntiMDA5 antibodypositive hypomyopathic dermatomyositis complicated with pneumomediastinum. Fukushima J. Med. Sci Vol. 64, No. 2, 2018
- 3. Morganroth PA, Kreider ME, Okawa J, Taylor L, Werth VP. Interstitial lung disease in classic and skin-predominant dermatomyositis: a retrospective study with screening recommendations. (2010) Archives of dermatology. 146 (7): 729-38. doi:10.1001/archdermatol.2010.134 - Pubmed