[Eosinophilic fasciitis (Shulman's syndrome)]

A 36-year-old man had noted a firm and painful swelling of the upper and lower limb, increasing over the previous few weeks. He had no fever, but the erythrocyte sedimentation rate was slightly increased (20 mm in the first hour). In addition there was a mild normochromic, normocytic anaemia (13 g/dl) and thrombocytosis (517,000/microliters). Gamma-globulin fraction was raised to 26%. The blood eosinophilia of 44% and the histological findings in a wedge biopsy of the lower leg (oedematous widened connective tissue septa, inflammatory infiltrate with eosinophilic granulocytes) established the diagnosis of eosinophilic fasciitis (Shulman syndrome). During oral treatment with prednisone (100 mg/d) the clinical symptoms regressed and the eosinophilia in peripheral blood disappeared. In the course of the subsequent seven months the prednisone dose was reduced gradually to 10 mg daily. But, because the symptoms did not entirely disappear the prednisone dose had to be increased temporarily for four times until there was a further, stepwise, reduction of the symptoms. But mild induration of the lower leg persisted. Maintenance treatment with prednisone, 10 mg daily, has been continued in order to avoid recurrence.