Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:P15088 (mast cell)
 14,925 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A twelve year-old boy who developed, after a period of strenuous physical work, an illness characterized by thickened skin over his right thigh and hemiabdomen, flexion contractures in right wrist and elbow and in right metacarpophalangeal joints without Raynaud's phenomenon or other visceral symptoms is presented. Pertinent laboratory studies showed hypereosinophilia and hypergammaglobulinemia. Deep-fascia biopsy showed typical findings of fasciitis with eosinophilia. Prednisone therapy resulted in sustained improvement. Periarticular osteopenia of the right hand and mast cell infiltration in fascial biopsy are remarkable features. Authors stress striking differences between fasciitis with eosinophilia or Shulman's syndrome and scleroderma. Clinical picture, laboratory changes, typical histology and a usually rapid response to corticosteroids, as well as some autoimmune diseases possibly associated, suggest a different disease and an immunological pathogenesis. From literature review authors conclude that cases of Shulman's syndrome reported in children are very limited and that this disease should by ruled out in every child with thickened skin changes and articular flexion contractures.
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PMID:[Fasciitis with eosinophilia: Shulman syndrome. Report of a case and review of the literature]. 305 3