再発性口腔内血管腫を契機に診断に至った青色ゴムまり様母斑症候群の1例

• Published in: 日本口腔外科学会雑誌 Vol. 66; no. 10; pp. 506 - 510
• Main Authors: 薮下, 雅子, 井口, 直彦, 吉田, 雅康, 吉田, 佳史, 佐藤, 一道, 野村, 武史
• Format: Journal Article
• Language: Japanese
• Published: 社団法人 日本口腔外科学会 20.10.2020
• Subjects: 口腔粘膜; 血管腫; 青色ゴムまり様母斑症候群
• ISSN: 0021-5163; 2186-1579
• DOI: 10.5794/jjoms.66.506

Blue rubber bleb nevus syndrome (BRBNS) is a rare disease characterized by multiple hemangiomas mainly in the skin and gastrointestinal tract. Here, we report a case of BRBNS due to recurrent intraoral hemangioma.　The patient was a 63-year-old woman who had recurrent tongue hemangioma and was referred to our department. Upper endoscopy revealed multiple hemangiomas in the stomach. We performed an excisional biopsy on the tongue lesion under general anesthesia, and a definitive diagnosis of cavernous hemangioma was established. The patient had a medical history of multiple hemangiomas of the fingers and a flare, and we finally made a definitive diagnosis of BRBNS. The patient was alive without recurrence or metastasis 40 months after surgery.　The first-line treatment for BRBNS is conservative treatment. The prognosis is poor in patients with gastrointestinal bleeding or central nervous system/circulatory system impairment. In the present case, there was no apparent gastrointestinal bleeding or invasion into other organs. Therefore, the patient had to be observed. However, in many cases, the lesions recur and develop during observation. Therefore, gastrointestinal examinations should be performed periodically. In addition, since some patients have multiple or recurrent intraoral hemangiomas as did our case, BRBNS should be diagnosed.