Skip Navigation LinksHome > August 2014 - Volume 36 - Issue 6 > Digestive Tract Symptoms in Congenital Langerhans Cell Histi...
Journal of Pediatric Hematology/Oncology:
doi: 10.1097/MPH.0b013e31829f35ad
Original Article

Digestive Tract Symptoms in Congenital Langerhans Cell Histiocytosis: A Fatal Condition in an Illness Usually Considered Benign

Vetter-Laracy, Susanne MD, PhD*; Salinas, Jose Antonio MD, PhD; Martin-Santiago, Ana MD; Guibelalde, Mercedes MD, PhD; Balliu, Pere-Ramon MD*

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Abstract

Introduction:

Congenital Langerhans cell histiocytosis is usually limited to cutaneous lesions and has a good prognosis. In rare cases of gut involvement, mortality is high and early and aggressive treatment essential.

Materials and Methods:

We report a case of histiocytosis in a newborn with bowel involvement, and performed a literature review of 13 similar cases worldwide documented between 1973 and 2008.

Results:

Skin eruptions are usually the initial symptoms at birth. Bloody stools or protein-losing enteropathy are the first signs of bowel involvement that appear mostly in the first 4 weeks of life. Risk organs (hematopoietic system, liver, spleen) are often affected in the newborns with intestinal Langerhans cell histiocytosis. Prognosis is usually poor, with 78.5% mortality.

Conclusions:

Even if histiocytosis in a neonate appears limited to autoinvoluting skin lesions, it is important to exclude all other organ involvement, including the bowel and stomach, as early treatment is vital.

Copyright © 2013 by Lippincott Williams & Wilkins

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