Epstein-Barr virus-positive primary cutaneous follicle centre lymphoma; an age-related phenomenon?

Primary cutaneous follicle centre lymphoma is the most frequently encountered primary cutaneous B cell lymphoma, often presenting in the head and neck region. Treatment is locally directed with surgery and/or radiotherapy. The prognosis is usually excellent with only local recurrences and generally no dissemination to extracutaneous sites. Epstein-Barr virus (EBV) is frequently encountered in lymphoproliferative disorders associated with immunosuppression, typically iatrogenic and congenital, or acquired as a consequence of a human immunodeficiency virus infection. More recently, age-related immune senescence has been proposed as a main driver in the evolution of EBV-positive lymphoproliferative disorders. We report a 50-year-old male presenting with a 2-3-year history of an inflamed eruption centred on the forehead and scalp. Incisional biopsy showed pathological features typical of primary cutaneous follicle centre lymphoma (PCFCL), and in the absence of systemic disease, he was treated with local radical radiotherapy. A cutaneous relapse occurred 10 months after initial diagnosis and 29 months after completing radiotherapy, he presented with a right-sided submandibular lymph node mass. Pathological examination of an excised node showed an EBV-positive diffuse large B cell lymphoma. Retrospective examination of the presenting PCFCL also showed the neoplastic lymphoid cells to be positive for EBV, both by in situ hybridization and on immunohistochemical staining for LMP1. EBV-associated PCFCL is an extremely rare occurrence. We speculate that, in this case, the EBV-positive lymphoma has most likely arisen as a consequence of age-related immune senescence, thereby further expanding the pathological spectrum of age-related EBV-associated B cell lymphoproliferations.