A Case of Letterer - Siwe Disease / 대한피부과학회지

ABSTRACT

Letterer-Siwe disease in an acutely onset, progressive and fatal disorder of RES, usually appears in male children, chareacterized by constitutional signs and extraos-seous lesions. This case occurred in a 18-day-old female showing hepatomegaly, hemorrhagic crusted papular eruptions on the entire body, and abdominal distension. Direct smear of scraping materials from the skin lesion showed numerous histiocytes. Hematologic and other laboratory examinatons showed no abonormalities.