Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of fasciitis with eosinophilia and hypergammaglobulinaemia (Shulman's syndrome) in a 33-year-old patient is described. Both the lower arms and the lower legs were affected. The diagnosis was established by biopsy which revealed cellular infiltration of the deep fascia with thickening. In addition to typical symptoms there was hepatosplenomegaly. Gastrointestinal or haematological disease was excluded. Under systemic corticosteroid administration all symptoms of fasciitis quickly disappeared.
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PMID:[Fasciitis with eosinophilia and hypergammaglobulinaemia (author's transl)]. 708 56

Eosinophilic fasciitis is a rare connective tissue disease in children characterized by hardening and thickening of the skin and soft tissues, peripheral eosinophilia, elevated erythrocyte sedimentation rate, and hypergammaglobulinemia. In this study, we report three pediatric patients with eosinophilic fasciitis whose clinical presentation and response to therapy differed from those reported in the literature. All three patients were female. Two of them had Raynaud's phenomenon as an initial feature, and the third had hepatosplenomegaly. Muscle involvement with weakness was present in two patients, as documented not only by increased aldolase levels at the time of presentation but also by inflammatory changes in the muscle biopsy. Serum immunoglobulin G levels were increased only in the most severely involved patient. Peripheral eosinophilia was present in all three cases. All three patients responded well to prednisone therapy. Two patients had a uni-phasic course, and one required further therapy to control a postinfection relapse. In summary, we found that Raynaud's phenomenon and hepatosplenomegaly can be part of the spectrum of clinical manifestations of childhood eosinophilic fasciitis. Identification as eosinophilic fasciitis and not scleroderma, despite hand involvement and Raynaud's phenomenon, can suggest that the illness may be steroid responsive.
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PMID:Faces of eosinophilic fasciitis in childhood. 1704 31