신경섬유종증 1형 가족력이 있는 환자에서 발생한 양성 두부 조직구증 1예

Benign cephalic histiocytosis is a rare subtype of non-Langerhans cell histiocytosis of unknown etiology. It usually occurs in infants and children and resolves spontaneously. A 14-month-old infant presented with multiple papules on his face, trunk, as well as upper and lower extremities. Histopathological examination of a biopsy specimen showed diffuse proliferation of histiocytes in the dermis with scattered lymphocytes and eosinophils. Immunohistochemical evaluation showed cells that were immunonegative for S-100 and CD1a but strongly immunopositive for CD68. Benign cephalic histiocytosis usually affects the head and neck areas and rarely spreads to other sites. Some reports in the available literature have described an association between juvenile xanthogranuloma and neurofibromatosis type 1; however, the association, if any, between benign cephalic histiocytosis and neurofibromatosis type 1 is unclear. We report a rare case of benign cephalic histiocytosis in a patient with a family history of neurofibromatosis type 1. (Korean J Dermatol 2021;59(1):58∼60)