The Association of Epstein-Barr Virus Infection and Hydroa Vacciniforme

摘要

Hydroa vacciniforme (HV) is one of the rarest usually quite severe photodermatosis of unknown etiology characterized by the childhood onset of necrotic vesiculopapules on exposed areas with resolution by early adult life Association with Epstein-Barr virus (EBV) infection and a possibly increased risk of lymphoproliferative malignancy have been raised Our study aims to determine the association of latent EBV infections and HV by clinical surveying the complications and outcomes of patients and evaluation of biopsy specimens A total of six patients of HV were retrospectively recruited from the dermatopathology database at National Cheng Kung University Hospital (NCKUH) during 1989 to 2009 The clinical features the findings of laboratory examinations and the skin biopsy specimens were retrospectively reviewed Clinically all six patients initially had recurrent vesicles necrotic ulcers or scars on sun-exposed areas (face or four extremities) The median age at diagnosis was 18 5 years and five of them were male The symptom onset time before diagnosis varied from one week to 10 years The mean follow-up time was 103 months per patient Four of six HV patients (66 67%) were positive for EBV infection by in situ hybridization technique among initial diagnostic skin biopsy specimens All of four EBV associated patients had relapsing course but only one of two EBER-negative patients relapsed Double staining of CD3 and EBV-encoded RNA (EBER) in situ hybridization proved EBV active infection in T cells Moreover one of EBV-associated HV patients had 209-month history of recurrent papulovesicular eruption spreading to non sun-exposed areas and died of HV-like T-cell lymphoma with hemophagocytosis To investigate the evolution of T cell lymphoma among HV patients we performed BIOMED-2 multiplex PCR methods for detecting T-cell clonality We gave up two patients’ specimens owing to their poor DNA quality and the T-cell clonality assay was performed in the biopsy specimens from the rest of four patients All initial biopsy specimens showed no T-cell monoclonality including the man who underwent 209-month HV progression had T-cell monoclonality in his final biopsy specimen and turned out to be HV-like T-cell lymphoma with hemophagocytosis Our series reveal older disease diagnosis age high EBV association rate and relatively aggressive disease course in the EBV associated patients Double staining of CD3 and EBER in situ hybridization proves EBV active infection in T cells Although HV is a rare type of photodermatosis clinical follow-up surveillance of active EBV infection and biopsy confirmation are necessary for possibly poor prognosis especially in EBV endemic areas To analyze T-cell clonality may be one of important ways to detect atypical HV progression and lymphoma changes

獎項日期	2014 8月 13
原文	English
監督員	Yu-Yun Lee (Supervisor)