Journal of the Korean Pediatric Society  1998;41(10):1424-1439

A Case of Congenital Self-Healing Reticulohistiocytosis.

Taek Sun KIM 1 ; Chang Wan KIM ; Eun Ryoung KIM ; You Mee KANG ; Kye Yong SONG

Affiliations

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Keywords

Congenital self healing reticulohistiocytosis; Langerhans cell; Birbeck granule; Laminated body

Country

Republic of Korea

Language

Korean

Abstract

Congenital self-healing reticulohistiocytosis (CSHR) is a rare variant of cutaneous reticulohistiocytosis. We report a newborn who had about 25 reddish brown papulonodular lesions on whole body at birth. No extracutaneous involvement was found. All skin lesions involuted within 4 months spontaneously. Biopsy of a skin lesion showed marked aggregated histiocytic infiltration with some eosinophils. S-100 protein and lysozyme were positive. Electron microscopy revealed numerous dense bodies, often with a myelin-like core and some Birbeck granules. We described a detailed clinical features of this case and reviewed the literatures.