Diffuse fasciitis with eosinophilia.

Abstract

The recently described syndrome of diffuse fasciitis with eosinophilia consists of scleroderma-like cutaneous changes in the absence of Raynaud's phenomenon and without visceral involvement. Peripheral blood eosinophilia and hypergammaglobulinemia occur in the majority of patients. The biopsy specimen is characteristic and shows a diffusely thickened fascia with a noticeable inflammatory infiltrate. The etiology and pathogenesis of the entity are unknown. The majority of the cases have been reported to respond to corticosteroids. Pulmonary function abnormalities and rheumatoid factor elevation in one of our patients suggests that this syndrome may be a variant scleroderma or may have previously unrecognized systemic manifestations. The patients' objective responses to corticosteroids were minimal.