Langerhans cell histiocytosis in a premature baby presenting with skin-isolated disease: case report and literature review

Authors

  • Shraga Aviner,

    1. Department of Pediatrics, The Barzilai Medical Center, Ashkelon, Israel
    2. Faculty of Health Sciences, The Ben-Gurion University of the Negev, Beer-Sheva, Israel
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  • Meir Ronen,

    1. Faculty of Health Sciences, The Ben-Gurion University of the Negev, Beer-Sheva, Israel
    2. Department of Dermatology, The Barzilai Medical Center, Ashkelon, Israel
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  • Daniel London,

    1. Faculty of Health Sciences, The Ben-Gurion University of the Negev, Beer-Sheva, Israel
    2. Radiology Department, The Barzilai Medical Center, Ashkelon, Israel
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  • Ana Tobar,

    1. Pathology Department, Rabin Medical Campus, Petach Tikva, Israel
    2. Sackler Faculty of Medicine, Tel-Aviv University, Ramat Aviv, Israel
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  • Shmuel Zangen

    1. Faculty of Health Sciences, The Ben-Gurion University of the Negev, Beer-Sheva, Israel
    2. Neonatal Intensive Care Unit, The Barzilai Medical Center, Ashkelon, Israel
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Correspondence
Shraga Aviner, M.D., Ph.D., Pediatric Day Care Center, The Barzilai Medical Center, 2 Hahistadrut Street, Ashkelon 78278, Israel. Tel: +972-8-674-6118 | Fax: +972-8-674-5165 | Email: aviners@barzi.health.gov.il

Abstract

Langerhans cell histiocytosis (LCH) in premature babies is extremely rare as is a vesicular skin rash, while gastrointestinal involvement is associated with a poor outcome. We report a case of LCH in a premature baby presented with isolated vesiculo-papulo-macular skin lesions and insidiously developed gastrointestinal symptoms, haematological and severe pulmonary involvement. We also reviewed a few cases of LCH in premature babies in the English language medical literature. LCH in preterm babies appears to be a severe systemic disease, usually lethal in-utero or post delivery.

Conclusion: Careful observation should be applied to newborns with skin-only Langerhans cell histiocytosis in order to identify in time progression to potentially fatal systemic disease.

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