Korean J Dermatol.
2023 Sep;61(8):513-517.
Benign Cephalic Histiocytosis in a Patient with Neurofibromatosis Type 1
- Affiliations
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- 1Department of Dermatology, Soonchunhyang University Bucheon Hospital, Soonchunhyang University College of Medicine, Bucheon, Korea
- 2Department of Dermatology, Soonchunhyang University Cheonan Hospital, Soonchunhyang University College of Medicine, Cheonan, Korea
Abstract
- Benign cephalic histiocytosis (BCH) is an uncommon subtype of non-Langerhans cell histiocytosis. A 5-month-old boy presented with multiple yellowish facial papules and plaques, which later spread to his trunk and both extremities. Laboratory tests, including lipid profile, were normal. Histological examination revealed non-foamy histiocytes, lymphocytes, and some eosinophils in the dermis. Immunohistochemical staining was positive for CD68 and factor XIIIa, but negative for CD1a and S-100. Additionally, the patient developed multiple café-au-lait spots with axillary and inguinal freckling. Next-generation sequencing identified a pathogenic variant of the neurofibromatosis type 1 (NF1) gene. Herein, we report a rare case of BCH in a patient with NF1. Although many cases of NF1 accompanied by juvenile xanthogranuloma have been reported, the association between BCH and NF1 has not been elucidated. However, considering that BCH may be a clinicopathological variant of juvenile xanthogranuloma, an association between the two diseases can be considered.
