[Shulman's syndrome: eosinophilic fasciitis (author's transl)]

A detailed report is made of the clinical, histological, biological manifestations of eosinophilic fasciitis, i. e. the Shulman's syndrome, about a 53-year-old man. An extreme induration of sub-cutaneous tissues from arms, legs and trunk, without involvement of the face and extremities, was associated with severe thickening of deep peri-muscular fascias. Raynaud's phenomenon was absent, as were morpheas and visceral involvement. Results of biopsies studied by standard, electron and I. F. 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 sub-cutaneous fat tissue. An elevated ESR, eosinophilia and hyperimmunoglobulinaemia 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 the Shulman's syndrome remain unclear. A critical review of the literature suggests that eosinophilic fasciitis should be separated from scleroderma and pseudoscleroderma, although this opinion has been discussed.