Epidermodysplasia verruciformis is a rare, multifactorial disorder.
The disease has genetic and immunologic components. Many patients
have impaired cell-mediated immunity. Although squamous
cell cancers may develop in EV patients, the association with other
hematological or solid malignancies and EV is reported in a few
studies. In this report, a case of EV with primary mediastinal large
B-cell lymphoma is presented.
A 20-year-old man with a five years history of red-brown macular
lesions was admitted to our hospital. The skin biopsy revealed
epidermodysplasia verruciformis. Persistent cough started during
the hospitalization. On the chest X-ray, mediastinal enlargement
was determined. Thoracic computed tomography demonstrated a
mediastinal mass of 6 cm in diameter. Diffuse, large B-cell lymphoma
was diagnosed. The chemotherapy (R-CHOP regiment) and
involved field radiotherapy were administered. Complete response
was achieved after the combined treatment modality. He is lymphoma-
free for 18 months follow-up.
Epidermodysplasia verruciformis is frequently seen in immunosuppressive
patients and many accompany infections with type human
papilloma virus subtypes such as 5 and 8. These human papilloma
virus subtypes that have oncogenic properties may trigger lymphomagenesis.
In addition, lymphomas are seen more frequently
in immunosuppressive patients than healthy persons. We conclude
that there may be a predisposition to lymphoma development in
epidermodysplasia verruciformis patients.