2017 Volume 127 Issue 4 Pages 615-620
We reported a case of chronic active Epstein-Barr virus infection (CAEBV) initially complicated by refractory facial edema. A 31-year old female noticed a facial swelling occurred nearly three months prior to visiting our clinic. When she visited us, her face, including both eyelids, was swollen and her lower legs showed an erythematous eruption without significant pruritus. Neither histological examination nor blood tests produced diagnostic findings. After close examinations by several departments in our hospital, a high fever followed by the onset of hemophagocytic syndrome (HPS) led us to consider the virus-related disorders, including CAEBV. Both an in-situ hybridization using skin biopsy tissues and a southern blotting assay of blood samples led us to diagnose CAEBV followed by HPS. After a few courses of chemotherapy, she moved to the university hospital to undergo bone marrow transplantation, but she died of intracranial hemorrhage due to a recurrence of HPS.
It is, therefore, suggested that a refractory facial edema could be an important initial symptom of CAEBV in adult patients and an in-situ hybridization method using skin biopsy specimens could provide a diagnostic clue for the virus-related disorders, especially for the diagnosis of life-threatening diseases such as CAEBV.
