EOSINOPHILIC FASCIITIS (SHULMAN SYNDROME) AS A RARE CAUSE OF SCLERODERMIFORM SKIN CHANGES

For 3 months a 70-year-old woman had been affected by painless, symmet rical swellings in the limbs, spreading proximally, causing hardening of the skin and impaired joint movements, but no fever. Her general co ndition and findings on physical examination were according to age. ES R was 22/42 mm and there was a thrombocytosis (533,000/mul) and eosino philia (16%, absolute 2,140/mul). Radiological and ultrasound examinat ions were normal. Skin biopsy revealed marked oedema of the connective tissue septa and perivascular lymphohistiocytic infiltrations, which - together with the other findings - suggested eosinophilic fasciitis. She was treated with prednisone, 100 mg daily, reduced gradually to 8 mg daily, as well as cimetidine, 800 mg daily, as immunomodulator, an d intensive physiotherapy. The swellings and eosinophilia regressed wi thin 8 weeks, but the hardening and impaired mobility persisted. Maint enance glucocorticoids are being continued.