SHULMANS SYNDROME - EOSINOPHILIC FASCIITIS

  • 1 January 1979
    • journal article
    • research article
    • Vol. 106  (11) , 859-866
Abstract
A detailed report is made of the clinical, histological and biological manifestations of eosinophilic fasciitis, or Shulman''s syndrome, in a 53 yr old man. An extreme induration of subcutaneous tissues from arms, legs and trunk, without involvement of the face and extremities, was associated with severe thickening of deep peri-muscular fasciae. Raynaud''s phenomenon was absent, as were morphea and visceral involvement. Results of biopsies studied by standard, electron and [immunofluoresence] microscopy, revealed sclerosis and cellular infiltrates (lymphocytes, plasma cells, histiocytes and eosinophils) in fascia and muscular septa; no changes were seen in epidermis, dermis or subcutaneous fat tissue. An elevated ESR [erythrocyte sedimentation rate], eosinophilia and hyperimmunoglobulinemia with high levels of circulating immune complexes were the only biological abnormalities. A good response to systemic corticosteroid therapy was observed. These features were similar to those seen in other cases of eosinophilic fasciitis. The etiology and pathogenesis of Shulman''s syndrome remain unclear. A review of the literature suggests that eosinophilic fasciitis should be separated from scleroderma and pseudoscleroderma.

This publication has 2 references indexed in Scilit: