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December 26, 2019

Degos-Like Cutaneous Findings in an Adult Woman With NXP-2–Associated Dermatomyositis

Author Affiliations
  • 1Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico–Milano, Milano, Italy
JAMA Dermatol. 2020;156(2):218-220. doi:10.1001/jamadermatol.2019.3940

A woman in her 20s was admitted to the intensive care unit of our hospital owing to the onset of severe dysphagia that required a nasogastric tube. Her symptoms had started 2 months previously with progressive myalgia, proximal muscle weakness, and fever followed by diffuse edema involving the face and extremities. Laboratory studies showed a peak creatine kinase level of 12 410 U/L (normal range, 26-240 U/L; to convert to microkatals per liter, multiply by 0.0167), an erythrocyte sedimentation rate of 66 (normal range, 1-20 mm/h), and a D-dimer level of 1639 μg/mL (normal range, <0.5 μg/mL; to convert to nanomoles per liter, multiply by 5.476). A screening autoimmune disease panel revealed positive results for antinuclear antibody (titer, 1:320; speckled granular pattern) and an anticardiolipin antibody level of 14 U/mL (normal range, <10 U/mL). Electromyography showed widespread acute muscle damage; deltoid muscle biopsy findings were consistent with necrotizing myopathy. Suspecting an autoimmune myopathy, we treated the patient with 4 sessions of γ-globulin intravenously (2 g/kg over a 5-day period) followed by oral prednisolone (65 mg/d) and mycophenolate mofetil (1000 mg twice a day), which had a partial benefit to her clinical conditions. After 2 months, she noticed worsening of her weakness and facial edema. In addition, she developed a violaceous eruption of the eyelids associated with small white spots on her face, chest, and arms.

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