Abstract
A case of diffuse fasciitis with eosinophilia is described. The case was clinically characterized by malaise, slight fever and diffuse swelling, pain and stiffness of the forearms and legs. The skin was sclerotic and bound down to the underlying structure. There was no sigh of Raynaud's phenomenon, nor internal organ involvement as seen diffuse scleroderma. The laboratory study showed marked eosinophilia in both peripheral blood and bone marrow and hypergamma-globulinemia (IgG 1,990 mg/dl). Hemoglobin, hematocrit, platelet counts, ESR and urinalysis was normal. Skin tests for various parasites, antinuclear antibody, anti-DNA antibody, antimicrosome antibody and LE test were all negative. The en bock biopsy of the forearm showed marked fascial thickening together with mononuclear and eosinophile infiltrates, but there was no pathologic change in the epidermis, dermis and muscle tissue. The diagnosis was based on these histologic changes and above clinical features. The response to corticosteroid therapy was remarkable. Since Shulman reported the first two cases in 1974, about 100 cases have been documented in the world literature. Our case represent the sixth case in Japan. Although the cause and pathogenesis of eosinophilic fasciitis remain unclear, there have recently been several case reports which suggest an autoimmune mechanism, such as association with autoimmune thrombocytopenic purpura and aplastic anemia. The present case did not have such hematological complication, but should be followed carefully.
