Langerhans cell histiocytosis: Diagnosis and Management

• DOI: 10.19100/jdvi.v4i1.161
• Publication Date: 2020-01-03T02:37:45Z
• Authors: Lusiana Lusiana; Hikari Ambara Sjakti; Githa Rahmayunita; Tjut Nurul Alam J...; Inge Ade Krisanti
• Keywords: Langerhans Cell Histiocytosis; Birbeck granules

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by abnormal clonal proliferation of dendritic cells. The incidence LCH 1 to 3 cases per million children worldwide, and occurs most frequently in 1–4 years age. etiopathogenesis LCH, whether it neoplastic or reactive, still controversial. has wide spectrum clinical features, dermatological abnormalities usually occur early. frequent lesions are elevated yellowish-red translucent papules about 1–2 mm diameter generally located seborrheic areas. recent classification (the Histiocyte Society study 2017) categorized into four groups; single system with involvement unifocal multifocal organs, lung multi-system either low- high-risk multiorgan involvement. definitive diagnosis typical morphology along Birbeck granules and/or positive results on CD1a antigen stain cells found lesions. Treatment multimodal, determined based age, extent lesion, organ involvement, location.