영아에서 진단된 다발성 부정형세포조직구증 1예

Abstract

Indeterminate cell histiocytosis is a rare disease of predominant cells having features that resemble both Langerhans cells and macrophages. The cells have greater pleomorphism with polyobated nuclei and these cells express markers for macrophages and Langerhans cells such as CD1a and S-100 protein, but absent Birbeck granules. We report a case of multiple indeterminate cell histiocytosis in a 3-month-old infant. He presents multiple reddish-brown, slightly yellowish papules on forehead, ear, shoulder, abdomen, testis and legs since 2 months earlier. The lesions were nonpruritic and painless with varied diameters of 2 mm to 2 cm. Immunohistochemically, the examination of biopsy revealed positive for macrophage marker (CD68) as well as for Langerhans cell markers such as S-100 protein and CD1a. No Birbeck granules were seen ultrastructurally in the cytoplasm of cells by electron microscopic study. So, it was confirmed as indeterminate cell histiocytosis. As we know, it is the first case in Korea which developed in neonatal period.