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Original Article

Digestive Tract Involvement with Exudative Enteropathy in Langerhans Cell Histiocytosis

, , , , &
Pages 515-524 | Received 15 Jul 1991, Accepted 06 Jan 1992, Published online: 09 Jul 2009
 

Abstract

Protein-losing enteropathy was observed in two children with Langerhans' cell histiocytosis (LCH). One patient was an infant with congenital cutaneous lesions; the second child had sigmoid and lymph node infiltration. Electron microscopy and immunohistochemistry confirmed, in both, infiltration of duodenum, skin, and liver by LCH. Gastrointestinal involvement by LCH seldom produces prominent clinical manifestations but indicates widespread multisystem disease. Immunohistochemual and/or ultrastructural features allow definitive diagnosis from mucosal biopsy specimens. Review of the literature of gastrointestinal infiltration by LCH emphasizes its poor prognosis, especially when associated with organ dysfunction.

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