Abstract

A 73-year-old female with a history of rheumatoid arthritis, treated with infliximab and methotrexate, presented for rapidly growing nodules on the face and arms. Physical examination showed a pink to purple plaque with central ulceration and eschar right upper forehead and a nodule present on the right nasal bridge and left forearm. Initial biopsy of the mass by outside institution revealed a diagnosis of EBV-positive/CD30-positive B-cell lymphoma with the differential diagnosis including EBV+ diffuse large cell B-cell lymphoma, grey zone lymphoma, and less likely, Hodgkin lymphoma. Due to concern for lymphoma, she was discontinued off of infliximab. Upon follow up visit, the patient noted improvement of lesions. Punch biopsy of a residual lesion revealed a perivascular and periadnexal lymphoid infiltrate composed predominantly of small cells with occasional large atypical cells, including rare forms with prominent nucleoli and binucleation. The large atypical cells were positive for CD15, CD30 and EBV and negative for CD3 and CD20. With improvement of the lesions after removal of immunotherapy, a diagnosis of an iatrogenic immunodeficiency-associated lymphoproliferative disorder was favored. Iatrogenic immunodeficiency-associated lymphoproliferative disorders, a distinct diagnostic category in the 2016 WHO, arise in patients treated with immunosuppressive drugs for autoimmune diseases. Methotrexate was the first reported immunosuppressive agent associated with lymphoproliferative disorders in this setting, predominately in those with RA. Histologically, Hodgkin lymphoma-like or diffuse large B-cell lymphoma-like features can be seen and there is often association with EBV. However, it is critical to distinguish this entity from other entities, as clinical improvement is observed upon removal of the immunosuppressive agent.

Financial Disclosure:
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