An otherwise healthy 6-year-old girl presented with 4 years of a rash on her hands, feet, elbows, and knees. Previously, she had used treatment with wet wraps and topical steroids without improvement. Her review of systems was notable for rare cough and dyspnea, but negative for muscle weakness. Physical examination revealed ragged cuticles; flat-topped pink papules overlying the bilateral metacarpophalangeal, proximal interphalangeal, and distal interphalangeal joints that were consistent with Gottron papules; and a spontaneous skin ulceration on her right knee (Figure). Her muscle strength was normal. Biopsy results of a metacarpophalangeal lesion revealed a lichenoid tissue reaction with basal vacuolation, which was consistent with dermatomyositis. Laboratory study results were notable for an antinuclear antibody titer of 1:320. Aldolase, creatine kinase, and myositis antibody panel results (Quest Diagnostics) were normal. Given the clinicopathologic features, a diagnosis of clinically amyopathic juvenile dermatomyositis (JDM) (CAJDM) was made. Treatment with hydroxychloroquine, prednisone, and clobetasol was administered while care was coordinated with pediatric pulmonology, rheumatology, and physical therapy departments. Ultimately, she was transitioned to receive treatment with methotrexate and intravenous immunoglobulin. At 1-year follow-up, myositis and interstitial lung disease remained absent and skin findings had improved.
Shawa H, Cotter DG. Clinically Amyopathic Juvenile Dermatomyositis. JAMA Dermatol. 2022;158(4):445. doi:10.1001/jamadermatol.2021.5310
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