Juvenile dermatomyositis associated to lymphoepithelioma, a nasopharyngeal carcinoma

Background Juvenile Dermatomyositis (JDM) is a systemic autoimmune inflammatory muscle disorder and vasculopathy that affects children younger than 16 years old. JDM was not associated with development of malignancies, unlike adult dermatomyositis. Nasopharynx carcinomas (NPC) are rare tumors, corresponding to 2% of tumors of the head and neck and 0,25% of all tumors. They are closely related to the Epstein Barr virus, and also genetic, environmental and viral factors. They occur predominantly among Asians (in certain regions inhabited by Chinese), males and in the range of 40 to 50 years of age. We report a 13-year-old female patient with juvenile DM associated with NPC. The relationship between Epstein-Barr virus infection and both DM and NPC suggests a possible viral cause. Our patient had an undifferentiated carcinoma associated with Epstein-Barr virus.