Langerhans cell histiocytosis: Presentation in a preterm neonate

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare disorder in which Langerhans cells (LC) accumulate in the skin or other organs and cause tumor formation or organ damage. Cutaneous lesions can vary widely and do not predict extent of systemic disease or prognosis. CASE: We present a premature infant with skin findings, multisystem involvement, and immunohistochemical markers consistent with multisystem LCH. CONCLUSION: Limited data from preterm neonates with LCH suggest that prognosis is particularly poor, with even limited cutaneous disease often rapidly progressing to become fatal, although diagnosis is not always prompt. Early diagnosis and treatment may affect prognosis.