Korean J Dermatol.  2021 Jan;59(1):58-60.

Benign Cephalic Histiocytosis with a Family History of Neurofibromatosis Type 1: A Case Report

  • 1Department of Dermatology, Hallym University Sacred Heart Hospital, Anyang, Korea


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.


Benign cephalic histiocytosis; Neurofibromatosis type 1; Non-Langerhans cell histiocytosis
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