Eosinophilic fasciitis (Shulman's syndrome):Case reports and review of the literature

Abstract

In 1974, Shulman et al. reported two patients with scleroderma-like skin changes, peripheral eosinophilia and hypergammaglobulinemia.1 This disorder was initially distinguished from progressive systemic sclerosis by the absence of Raynaud's phenomenon, visceral involvement, and a favorable response to corticosteroid therapy. Histologically, there was marked thickening of the fascia with striking inflammation.1 The term eosinophilic fasciitis (EF) was suggested by Rodnan et al.due to the presence of the striking peripheral eosinophilia and the finding of large numbers of these cells in the inflamed deep fascia and subcutis.2 However, further studies showed that eosinophils are not a consistent component of the inflammatory infiltrate in the fascia and can be absent.3 One patient with localized eosinophilic fasciitis from Saudi Arabia has been reported.4 In this paper, we further report 2 cases with generalized EF. The etiology of Shulman's syndrome is still unknown, although a hypersensitivity reaction and autoimmune processes have been suggested.