Abstract
BACKGROUND: Extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT), is a rare, aggressive subtype of non-Hodgkin lymphoma, and it usually presents as a destructive sinus mass that may attend epistaxis. However, ENKTCL-NT with the manifestation of peripheral neuropathy is pretty unusual. CASE PRESENTATION: A 17-year-old Chinese patient presented with peripheral neuropathy and positive auto-antibodies. However, she failed methylprednisolone pulse therapy and developed hemophagocytic syndrome. The diagnosis of CD30-positive primary cutaneous ENKTCL-NT was confirmed by pathological biopsy. Her disease was brought under control after five cycles of chemotherapy. CONCLUSIONS: The report findings are helpful in the differential diagnosis of peripheral neuropathy and autoimmune disease. We should be alert for the development of ENKTCL-NT when the rash and peripheral neuropathy are the first symptoms and are accompanied by Epstein-Barr virus (EBV) infection.