[Shulman's syndrome: fasciitis with eosinophilia, pseudoscleroderma with eosinophilia].
The authors report two cases of the Shulman's syndrome. Twenty one cases of this new entity are found in the literature. The main signs are the acute painful onset after an unusual physical exertion, the development of fasciitis, and (or) myositis, and (or) scleroderma of limbs, the absence of Raynaud's phenomenon, visceral involvement, constant eosinophilia and hypergammaglobulinemia; the prognosis is good with an improvement sometimes spontaneous or coincident with prednisone therapy. One of the observations is interesting by the association with Gougerot-Sjogren's syndrome, and a familial case of morphea. The prognosis in this case is mediocre: failure of corticotherapy, and incomplete remission with cyclophosphamide.
D004802:Eosinophilia / Q000150:complications*; D005205:Fascia; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications*; D008297:Male; D008875:Middle Aged; D009132:Muscles / Q000473:pathology; D009220:Myositis / Q000150:complications*; D012595:Scleroderma, Systemic / Q000145:classification / Q000175:diagnosis / Q000473:pathology; D012867:Skin / Q000473:pathology; D013577:Syndrome
Le syndrome de shulman: fasciite avec eosinophilie; pseudo-sclérodermie a éosinophiles. A propos de 2 cas.