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Eosinophilic fasciitis (EF) is a disorder in which the subcutaneous tissues become indurated and then sclerotic leading to myalgia, arthralgia, and occasionally contractures and disability. High-dose systemic steroids are first-line therapy; however, steroid-sparing agents are often used owing to disease progression or to avoid the adverse effects of long-term corticosteroid use. We describe a case of EF with rapid response to sirolimus.
A man in his 30s had a 6-month history of arthralgia involving his shoulders, hands, knees, ankles, and feet, which made it difficult for him to stand or walk for long periods. He complained of swelling and “skin tightness” of his arms, lower legs, and hands. On examination, he had woody induration of his distal extremities creating both peau d’orange surface change and linear depressions along the veins of his right forearm consistent with a groove sign. His fingers were held in a slight flexion contracture at the proximal interphalangeal joints (Figure, A).
Oza VS, Walsh R, North J, Berger TG, Murase JE. Treatment of Eosinophilic Fasciitis With Sirolimus. JAMA Dermatol. 2016;152(4):488-490. doi:10.1001/jamadermatol.2016.0048