선천성 자연치유성 망상 조직구 증식증 1예

Abstract

Congenital self healing reticulohistiocytosis was described by Hashimoto and Pritzker in 1973. Hundreds of cases were reported in America and Europe, but cases reported in Korea are rare especially in cases with multiple skin lesions. We present a case of multiple congenital self healing reticulohistiocytosis in a full termed, normal spontaneous vagina1 delivered male neonate. He had erythematous to reklish colored nodules or bullae on his whole body and some of them were hemorrhagic in its appearance. The oral and anogenital mucosae had no lesions. Laboratory tests showed no evidence of intrauterine infections and systemic involvement. The histopathologic examination revealed densley aggregated dermal histiocytes and an absence of epidermis, Infiltrated histiocytes showed a positivity for S-100 protein. About 15% of infiltrated cells had Birbeck granules on an electron microscopic study, Four months later, the skin lesions cleared and there was no evidence of systemic involvement.