Abstract
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.
| Original language | English |
|---|---|
| Pages (from-to) | 1102-1111 |
| Number of pages | 10 |
| Journal | Journal of Cutaneous Pathology |
| Volume | 43 |
| Issue number | 12 |
| DOIs | |
| Publication status | Published - 2016 Dec 1 |
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All Science Journal Classification (ASJC) codes
- Pathology and Forensic Medicine
- Histology
- Dermatology
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Hydroa vacciniforme and hydroa vacciniforme-like T-cell lymphoma : an uncommon event for transformation. / Chen, Chien Chin; Chang, Kung Chao; Medeiros, L. Jeffrey; Lee, Julia Yu Yun.
In: Journal of Cutaneous Pathology, Vol. 43, No. 12, 01.12.2016, p. 1102-1111.Research output: Contribution to journal › Article
TY - JOUR
T1 - Hydroa vacciniforme and hydroa vacciniforme-like T-cell lymphoma
T2 - an uncommon event for transformation
AU - Chen, Chien Chin
AU - Chang, Kung Chao
AU - Medeiros, L. Jeffrey
AU - Lee, Julia Yu Yun
PY - 2016/12/1
Y1 - 2016/12/1
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=84988009694&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84988009694&partnerID=8YFLogxK
U2 - 10.1111/cup.12801
DO - 10.1111/cup.12801
M3 - Article
C2 - 27531667
AN - SCOPUS:84988009694
VL - 43
SP - 1102
EP - 1111
JO - Journal of Cutaneous Pathology
JF - Journal of Cutaneous Pathology
SN - 0303-6987
IS - 12
ER -