EBV positive mucocutaneus ulcer: A manifestation of lymphoproliferative disorders in the head and neck area

Introduction The EBV positive mucocutaneus ulcer (EBV MCU) was recently classified as a new entity of the cutaneous lymphoproliferative B-cell disorders by the WHO. Clinically self-limiting solitary ulcers of mainly the oropharyngeal or gastrointestinal mucosa are present. Aetiological decisive is an immunosuppression additive to a latent EBV - infection. Worldwide there are 52 described cases.

Methods Case report of a 90-year-old patient with painful, therapy resistant ulcers in the hypopharynx and at the bottom lip as well as a swelling of the neck lymph nodes. The patient also has a history of chronic lymphocytic leukaemia (CLL). Tissue biopsies of the ulcers and the swollen lymph nodes were performed.

Results Histopathologically and immunohistochemically the tissue samples of the ulcers showed an increased number of CD 30 - blasts, which could be morphologically distinguished from Hodgkin- respectively Sternberg-Reed cells. Additionally, the in-situ hybridization (EBER) showed a noticeable EBV activation. Therefore, in combination with the clinical aspects an EBV- positive lymphoproliferative disorder of the entity EBV MCU could be diagnosed. The biopsy of the lymph nodes showed a CLL.

Discussion Since the EBV MCU is a so far relatively unknown entity of the cutaneous lymphoproliferative disorders, it has to be considered as a differential diagnosis in elderly and immunocompromised patients with therapy resistant ulcers of the mucosa. Furthermore, the histopathological differentiation from other malignant disorders is crucial.

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Publication History

Article published online:
10 June 2020