Recurrent scarring papulovesicular lesions on sun-exposed skin in a 22-year-old man

Levoska, Melissa A.; Cohen, Jeffrey I.; Manoli, Irini; Lee, Chyi‐Chia Richard; Ching, Steven; Shand, Jessica C.; Tamura, Deborah; Kraemer, Kenneth H.; DiGiovanna, John J.

doi:10.1016/j.jaad.2017.08.005

Cited by 4 publications

(2 citation statements)

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“…Patients with HVLPD were defined as having papulovesicular skin lesions in sun-exposed areas that healed with scarring and markedly elevated levels of EBV DNA (.10 000 copies/mL) in the blood with high levels of EBV DNA in T or NK cells. Patients 1, 10 11, 11 and 13 12 were reported previously. Patients with classic HVLPD had no persistent systemic symptoms, lymphadenopathy, hepatosplenomegaly, hepatitis, hemophagocytic syndrome, or NK-cell lymphocytosis, while those with systemic HVLPD had $1 of these persistent symptoms or signs of extracutaneous disease (based on prior criteria 6 ).…”

Section: Patientsmentioning

confidence: 93%

Hydroa vacciniforme–like lymphoproliferative disorder: an EBV disease with a low risk of systemic illness in whites

Cohen

Manoli

Dowdell

et al. 2019

Blood

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Patients with classic hydroa vacciniforme–like lymphoproliferative disorder (HVLPD) typically have high levels of Epstein-Barr virus (EBV) DNA in T cells and/or natural killer (NK) cells in blood and skin lesions induced by sun exposure that are infiltrated with EBV-infected lymphocytes. HVLPD is very rare in the United States and Europe but more common in Asia and South America. The disease can progress to a systemic form that may result in fatal lymphoma. We report our 11-year experience with 16 HVLPD patients from the United States and England and found that whites were less likely to develop systemic EBV disease (1/10) than nonwhites (5/6). All (10/10) of the white patients were generally in good health at last follow-up, while two-thirds (4/6) of the nonwhite patients required hematopoietic stem cell transplantation. Nonwhite patients had later age of onset of HVLPD than white patients (median age, 8 vs 5 years) and higher levels of EBV DNA (median, 1 515 000 vs 250 000 copies/ml) and more often had low numbers of NK cells (83% vs 50% of patients) and T-cell clones in the blood (83% vs 30% of patients). RNA-sequencing analysis of an HVLPD skin lesion in a white patient compared with his normal skin showed increased expression of interferon-γ and chemokines that attract T cells and NK cells. Thus, white patients with HVLPD were less likely to have systemic disease with EBV and had a much better prognosis than nonwhite patients. This trial was registered at www.clinicaltrials.gov as #NCT00369421 and #NCT00032513.

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Section: Patientsmentioning

confidence: 93%

Hydroa vacciniforme–like lymphoproliferative disorder: an EBV disease with a low risk of systemic illness in whites

Cohen

Manoli

Dowdell

et al. 2019

Blood

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“…Hydroa vacciniforme (HV)-like lymphoproliferative diseases (HVLPD) are chronic Epstein-Barr virus (EBV)-related diseases that manifest with characteristic vesiculopapular, edema, ulcer, and scars, called HV eruption [1][2][3]. HVLPD includes classic HV, severe HV, and HV-like T-cell lymphoma (HVLL).…”

Section: Introductionmentioning

confidence: 99%

Clinicoprognostic Study of Hydroa Vacciniforme-like Lymphoproliferative Diseases: A Systematic Review

Kim¹,

Yang²,

Won³

et al. 2023

Dermatology

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Background: Limited clinicopathological and prognostic data are available on hydroa vacciniforme (HV)-like lymphoproliferative diseases (HVLPD). Methods: This systematic review searched HVLPD reports in Medline via PubMed, Embase, Cochrane, and CINAHL databases in October 2020. Results: A total of 393 patients (65 classic HV, 328 severe HV/HV-like T-cell lymphoma [HVLL]) were analyzed. Among severe HV/HVLL cases, 56.0% were Asians, whereas 3.1% were Caucasians. Facial edema, hypersensitivity to mosquito bites, the onset of skin lesion, and percentage of severe HV/HVLL differed significantly by race. Progression to systemic lymphoma was confirmed in 9.4% of HVLPD patients. Death occurred in 39.7% patients with severe HV/HVLL. Facial edema was the only risk factor associated with progression and overall survival. Mortality risk was higher in Latin Americans than in Asians and Caucasians. CD4/CD8 double-negativity was significantly associated with the worst prognosis and increased mortality. Conclusion: HVLPD is a heterogeneous entity with variable clinicopathological features associated with genetic predispositions.

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2019

Longitudinal Observation of Pediatric Dermatology Patients

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Recurrent scarring papulovesicular lesions on sun-exposed skin in a 22-year-old man

Abstract: KEY TEACHING POINTS.

Cited by 4 publications

References 45 publications

Hydroa vacciniforme–like lymphoproliferative disorder: an EBV disease with a low risk of systemic illness in whites

Hydroa vacciniforme–like lymphoproliferative disorder: an EBV disease with a low risk of systemic illness in whites

Clinicoprognostic Study of Hydroa Vacciniforme-like Lymphoproliferative Diseases: A Systematic Review

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