EBV陽性皮膚粘膜潰瘍との鑑別を要したEBV陽性リンパ増殖性疾患例

A case of Epstein-Barr virus-positive lymphoproliferative disorder requiring differentiation from Epstein-Barr virus-positive mucocutaneous ulcer

はじめに

　エプスタイン・バーウイルス（Epstein-Barr virus：EBV）陽性皮膚粘膜潰瘍（以下，EBV-MCU）は，皮膚や粘膜に限局性の潰瘍性病変を生じ，組織学的にはReed-Sternberg細胞に類似したEBV陽性の異型B細胞の増殖を特徴とする疾患である^1）。免疫組織学的には，異型細胞はCD20陽性・CD30陽性の活性化Bリンパ球の特徴を示すことが多い^2）。EBV-MCUは予後良好な疾患であり，再発や増悪を示すことは稀である。今回われわれは，EBV-MCUとして治療されたのち，経過観察中に全身のリンパ節腫脹からEBV陽性リンパ増殖性疾患（EBV-LPD）と診断され不幸な転帰をたどった症例を経験したので報告する。

　We present a case of Epstein-Barr virus-positive mucocutaneous ulcer（EBV-MCU）that manifested on the oropharyngeal mucosa.

　The patient was a 70-year-old man with a sore throat who exhibited ulcers in the oropharyngeal mucosa. Initial biopsy results showed that the morphologic and immunophenotypic features of the oropharyngeal ulcer were consistent with EBV-MCU. Three months of treatment with prednisolone healed the oropharyngeal ulcer. However, after nine months of treatment, the patient developed generalized lymphadenopathy and received a diagnosis of EBV-LPD. Despite being treated with R-CHOP, the patient died owing to EBV-LPD progression.

　Clinical evaluations are crucial in distinguishing EBV-LPD from EBV-MCU. When diagnosing EBV-MCU, systemic examinations and blood tests should be conducted with the possibility of detecting EBV-LPD.