The Shulman syndrome: report of a further case

A 53-year-old woman developed swelling of her hands after strenuous sport. Soon her feet became affected and the swelling spread up the arms and legs making arm movement and walking difficult. No Raynaud's phenomenon appeared or other features of systemic sclerosis. There was eosinophilia and hypergammaglobulinaemia in the peripheral blood. Histological examination of the skin showed an increase in the collagen bundles in the dermis extending down to the adipose tissue. The fascia showed dense fibrous tissue with a perivascular infiltrate of lymphocytes, a few plasma cells and eosinophils. Immunohistology of the skin and fascia were negative for IgA, IgG, IgM, C3, CIq and fibrin. After 4 weeks of prednisolone 40 mg daily there was a great improvement. This continued with further corticosteroid therapy.