Liver Involvement in Langerhans Cell Histiocytosis

Abstract

Langerhans cell histiocytosis (LCH), is an uncommon disease in children, characterized by the proliferation and inflammatory neoplasia of myeloid dendritic cells with varied clinical presentations. The disease is divided into a single system or multisystem. Multisystem form usually affects organs like the bones, skin, liver, spleen, lungs and central nervous system. Cutaneous lesions were one of the most common manifestations, and were seborrheic dermatitis -like lesions can have diagnosis of LCH is often difficult and delayed. Liver involvement usually presents as a part of the disseminated process but the clinical features remain poorly defined. Early diagnosis of the disease and treatment respond better to therapy. Here we report a case of LCH with liver involvement, after first chemotherapy the patient was lost to follow-up with further worsening of symptoms. Continuing chemotherapy was planned but the general condition of the patient deteriorated and he died due to acute bronchopneumonia.