A Case of Letterer: Siwe Disease / 대한피부과학회지

ABSTRACT

A 5-month-old male infantshowed greasy sealy papules on the scalp, dirty scaly papules on the trunk and scaly petechial papules on both the palms and soles since birth. When admitted to the hospital, the patient exhibited lymphadenopathy, hepatomegaly, abdominal distension, mild fever and skin eruption. The biopsy specimens from the skin and lymph node showed an infiltrate composed almost entirely of histiocytic cells with irregularly shaped nuclei and abundant, well demarcated cytoplasm. The histiocytic cells of the infiltrate were proved as Langerhans cells by electron microscopic examination and S-100 protein study. He was treated with methotrexate 30mg/m twice a week and prednisolone 40mg/m daily. Initially skin lesions and general condition were improved, but the patient expired. due to septicemia after 3 months.