口蓋の潰瘍を契機に診断されたエルドハイム・チェスター病の1例

Abstract

We report a case of Erdheim-Chester disease diagnosed as a result of ulceration of the palate. The patient was a 62-year-old male with the chief complaint of contact pain of the palatal mucosa. He had a beaded mass on the adductor muscle of the right thigh, which was being treated by an orthopedic surgeon. At the same time, an ulcerative lesion was found on the palate. He was referred to the Department of Oral and Maxillofacial Surgery and treated with a steroid ointment. However, the lesion did not improve and a biopsy was performed two weeks later. The biopathologic finding was similar to that of the soft tissue tumor of the leg, and finally a diagnosis of Erdheim-Chester disease was made. The oral lesions did not improve and the contact pain persisted, so protection of the ulcerated surface was performed with indomethacin spray and a 0.5mm celluloid splint. The contact pain was alleviated and feeding improved. However, although chemotherapy was performed for the tumors in both legs, the treatment was discontinued and palliative care was initiated since his general condition worsened due to the exacerbation of the primary disease, hepatocellular carcinoma.