Langerhans Cell Histiocytosis Presenting as Enterocolitis and Shock in Neonate

INTRODUCTION: Enterocolitis is a relatively common disease in neonatal period that can be a result of many underlying pathologies. One of them, which is an unusual disorder especially in neonatal age and with gastrointestinal involvement, is Langerhans cell histiocytosis (LCH). This case shows a severe neonatal LCH with digestive involvement which required intensive care and had an abnormal presentation, being hard to diagnose attributable to the diversity of symptoms. CASE REPORT: Eleven-day-old newborn presented for excessive weight loss followed by deterioration to shock, abdominal distension, digestive bleeding, and purpuric exanthema. Exploratory laparotomy identified aggressive enterocolitis. After stabilization, a significant hepatosplenomegaly persists as well as bicytopenia, pyrexia, and cutaneous lesions evolving tangible purple. LCH was diagnosed through histology of cutaneous biopsy. CONCLUSIONS: Gastrointestinal involvement in neonatal LCH is infrequent and its symptoms can be really unspecific. It is important to know that the first clinical manifestation is usually dermatologic with very diverse morphologies. Having a high suspect rate will lead us to an early diagnosis with its correspondent impact upon the outcome.