6か月男児の下顎骨と体幹皮膚に生じた多臓器多病変型ランゲルハンス細胞組織球症の1例

• Published in: 日本口腔外科学会雑誌 Vol. 66; no. 9; pp. 439 - 443
• Main Authors: 渋谷, 恭之, 中村, 知寿, 森下, 雄斗, 鍋田, 剛志, 加藤, 伸一郎, 福島, 麻子
• Format: Journal Article
• Language: Japanese
• Published: 社団法人 日本口腔外科学会 20.09.2020
• Subjects: 6か月男児; ランゲルハンス細胞組織球症; 下顎骨; 体幹皮膚
• ISSN: 0021-5163; 2186-1579
• DOI: 10.5794/jjoms.66.439

Langerhans cell histiocytosis (LCH) is a relatively rare proliferative disorder of Langerhans cells. We herein report an extremely rare case of LCH arising in the mandible and trunk skin of a 6-month-old boy. A patient was referred to our clinic because of right cheek swelling. Computed tomography revealed broad bone expansion and a defect in the right mandibular molar region. An excisional biopsy was performed, and the lesion was histopathologically diagnosed as LCH (multi-system multi-site: MM type). Immunohistochemically, the histiocytes were positive for S -100 and CD1a. The patient subsequently underwent chemotherapy (vinblastine and prednisolone), and the lesion was reduced completely. Long-term follow-up should be practiced to monitor for tumor recurrence and hypoplasia of the teeth as a side effect of chemotherapy. There has been no recurrence after 2 and a half years of follow-up.