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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*

Journal of Pediatric Hematology/Oncology:
doi: 10.1097/MPH.0b013e31829f35ad
Original Article

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.

Author Information

*Department of Pediatrics, Division of Neonatology

Department of Pediatrics, Division of Oncology

Department of Dermatology, Hospital Universitari Son Espases, Palma de Mallorca, Spain

The authors declare no conflict of interest.

Reprints: Susanne Vetter-Laracy, MD, PhD, Department of Pediatrics, Division of Neonatology, Hospital Universitari Son Espases, Carretera Valldemossa 79, Palma de Mallorca 07120, Spain (e-mail:

Received April 19, 2013

Accepted May 8, 2013

© 2014 by Lippincott Williams & Wilkins.