Systemic lupus erythematosus after eosinophilic fasciitis: A case report

Abstract

Eosinophilic fasciitis (EF) is an uncommon connective tissue disease characterized by scleroderma-like cutaneous changes, peripheral eosinophilia, hypergammaglobulinemia, and an elevated erythrocyte sedimentation rate. Typical histologic findings include a chronic inflammatory infiltrate affecting the deep fascia with lymphocytes, hystiocytes, and, occasionally, eosinophils.1 Whether EF is a separate entity or a variant of scleroderma is unclear.2 Visceral involvement is infrequent although isolated cases of pulmonary, esophageal, and cardiac involvement have been reported.3 Arthritis, low-grade myositis, and carpal tunnel syndrome are the most common other manifestations attending EF.3,4 An aberrant immune response has been proposed for the pathogenesis of EF, because most diseases related to EF have an autoimmune basis. However, malignant transformation in hematopoietic cells has also occurred.5 EF responds to prednisone or hydroxychloroquine. The prognosis is usually excellent, except when it is associated with a malignant hematologic disease. We describe a patient with EF who, 8 years later, experienced the development of systemic lupus erythematosus (SLE). To our knowledge, only two previous cases of EF associated with SLE have been reported.