Hydroa vacciniforme and hydroa vacciniforme-like T-cell lymphoma: an uncommon event for transformation

• Published in: Journal of cutaneous pathology Vol. 43; no. 12; pp. 1102 - 1111
• Main Authors: Chen, Chien-Chin, Chang, Kung-Chao, Medeiros, L. Jeffrey, Lee, Julia Yu-Yun
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01.12.2016
• Subjects: Adolescent; Adult; Cell Transformation, Viral; Child; Epstein-Barr virus; Epstein-Barr Virus Infections - complications; Epstein-Barr Virus Infections - epidemiology; Epstein-Barr Virus Infections - pathology; Female; Herpesvirus 4, Human; Humans; HV-like T-cell lymphoma; hydroa vacciniforme (HV); Hydroa Vacciniforme - pathology; Hydroa Vacciniforme - virology; Immunohistochemistry; In Situ Hybridization; Lymphoma, T-Cell - virology; Male; Polymerase Chain Reaction; T-cell clonality; transformation; Young Adult; Epstein-Barr virus; transformation; HV-like T-cell lymphoma; hydroa vacciniforme (HV); T-cell clonality
• ISSN: 0303-6987; 1600-0560
• DOI: 10.1111/cup.12801

Background Hydroa vacciniforme (HV) is associated with Epstein‐Barr virus (EBV) infection and a risk of transformation to lymphoma. Methods We retrospectively analyzed six HV cases for EBV association and transformation to HV‐like T‐cell lymphoma. Clinicopathologic features were reviewed and cases were assessed for EBV‐encoded RNA (EBER) by in situ hybridization, double staining with immunohistochemistry and EBER and for T‐cell clonality. Results The male‐to‐female ratio was 5:1, with a median age at diagnosis of 18.5 years. All patients initially had recurrent vesicles, necrotic ulcers or scars on sun‐exposed areas. Symptoms were present before diagnosis between 2 weeks to 10 years. The mean follow‐up time was 106.3 months. Four patients (67%) were EBV‐positive. All four EBV‐positive and one EBV‐negative patients had relapsing clinical course. Double staining proved EBV infection in T‐cells. Moreover, one EBV‐positive patient developed HV‐like T‐cell lymphoma with hemophagocytosis after 209 months of recurrent papulovesicular eruptions and eventually died. T‐cell clonality was successfully performed in four HV patients and all showed polyclonal results; the transformed HV‐like T‐cell lymphoma was monoclonal. Conclusions In EBV endemic areas, HV is frequently (67%) associated with EBV infection, but transformation to HV‐like T‐cell lymphoma seems to be uncommon (17%) and bear a dismal outcome.