, Volume 4, Issue 1, pp 81-86
Date: 18 Dec 2007

Anti-neutrophil Cytoplasmic Antibody (c-ANCA) Positive Recurrent Eosinophilic Fasciitis Responsive to Cyclophosphamide

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Case presentation

A 65-year-old white man who carried the diagnosis of eosinophilic fasciitis (EF) presented with worsening neck, shoulder, and back stiffness over the last 3 years. His symptoms started 8 years ago with abdominal muscle tightness and skin thickness, which progressed to upper and lower extremities within weeks. During this period, he also complained of exertional muscle pain, especially after prolonged exercise. Based on peripheral eosinophilia and a muscle biopsy, he was diagnosed with EF and treated successfully with high-dose corticosteroids (CS). However, each attempt to decrease CS dose below 20 mg resulted in worsening of his muscle tightness, especially around proximal upper extremities and neck muscles. Five years before the presentation, he used hydroxychloroquine for 8 months and methotrexate for another 8 months as steroid tapering agents, with no benefit. Three years before the presentation, he developed CS myopathy and thus CS was stopped. Six months before ...