A Case of Neonatal Langerhans Cell Histiocytosis Presenting with Generalized Skin Involvement at Birth Evolving to Hepatic Involvement

Abstract

Langerhans cell histiocytosis (LCH) is a rare disease having an unknown etiology, but characterized by a disorder of antigen presenting cells and the mononuclear phagocytic system. Neonatal LCH, defined as LCH presenting within the first 4 weeks of life, accounts for less than 5-6% of the total LCH cases. Skin involvement of neonatal LCH tends to regress spontaneously, as has been shown in Hashimoto-Pritzker syndrome. We report a case of LCH that presented as whole body, polymorphic, generalized, erythematous, yellowish plaques at birth, with no evidence of other organ involvement. These skin lesions regressed spontaneously within 14 days. Two weeks later, however, the patient presented with hepatosplenomegaly, fever, hyperbilirubinemia, anemia and leukocytosis. LCH with liver involvement was shown on abdominal MRI findings, and the patient received systemic chemotherapy. Although neonatal LCH may initially only show skin manifestations, it should be followed up closely and periodically for other organ involvement.