Dermatology grand rounds at the NIH
Recurrent scarring papulovesicular lesions on sun-exposed skin in a 22-year-old man

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Key teaching points

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    Typical HV is a self-limited EBV-associated disorder of children and adolescents characterized by recurrent outbreaks of scarring papulovesicular lesions. The criterion standard diagnostic test is biopsy of an active lesion showing reticular degeneration and demonstrating EBV RNA (EBER positivity) in lymphocytes.

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    Avoiding sun exposure by using photoprotection can reduce outbreaks.

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    Patients typically have high levels of circulating EBV DNA, which is best detected from whole blood rather than from serum or plasma.

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    In patients with persistent skin disease activity, following EBV DNA levels in the blood can help assess for the development of aggressive systemic disease.

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    Severe HV disease associated with chronic active EBV may progress to lymphoproliferative disorders or lymphoma with increased mortality.

Section snippets

History

A 22-year-old white man has been followed in the Clinical Center at the National Institutes of Health (NIH) for recurrent outbreaks of scarring herpetiform lesions on sun-exposed areas. The patient and/or parents signed consent forms and were followed under NIH protocols that were approved by the institutional review boards of the National Human Genome Research Institute, National Institute of Allergy and Infectious Diseases, and National Cancer Institute.

He reported itching and burning within

Discussion

HV is a rare childhood photodermatosis associated with latent EBV infection.1 Typical HV has outbreaks that begin during childhood or early adolescence without systemic involvement and usually resolve by young adulthood.2, 3 Some cases persist into adulthood with elevated levels of EBV in the blood.4, 5 Most cases of typical HV have been diagnosed in the United States, Europe, Australia, South Africa, and Asia.5, 6, 7, 8 In contrast, patients with severe HV-like eruptions are often from Asia9,

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    • Cited by (4)

    • Hydroa vacciniforme-like lymphoproliferative disorder: An EBV disease with a low risk of systemic illness in whites

      2019, Blood
      Citation Excerpt :

      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 1312 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 criteria6).

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

      2023, Dermatology

    • Actinic Prurigo and Hydroa Vacciniforme

      2019, Current Dermatology Reports

    • Longitudinal Observation of Pediatric Dermatology Patients

      2019, Longitudinal Observation of Pediatric Dermatology Patients

    Supported by the Intramural Research Programs of the Center for Cancer Research, National Cancer Institute, National Institute of Allergy and Infectious Diseases, and National Human Genome Research Institute, National Institutes of Health (NIH). Support for Ms. Levoska was made possible through the NIH Medical Research Scholars Program, a public-private partnership supported jointly by the NIH and generous contributions to the Foundation for the NIH from the Doris Duke Charitable Foundation, the American Association for Dental Research, the Colgate-Palmolive Company, Genentech, and other private donors. For a complete list, visit the foundation website at http://www.fnih.org.

    Conflicts of interest: None disclosed.

    Reprints are not available from the authors.

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    © 2017 by the American Academy of Dermatology, Inc.