Hydroa Vacciniforme

Updated: Jun 14, 2021
  • Author: Gregory Toy, MD; Chief Editor: Dirk M Elston, MD  more...
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Hydroa vacciniforme (HV) is a rare, chronic photodermatosis of unknown origin occurring in childhood that exists within the spectrum of Epstein-Barr virus–related lymphoproliferative disorders. These range from classic HV to fatal lymphoma. [1]

Recurrent vesicles on sun-exposed skin that heal with vacciniform or varioliform scarring characterize HV. The histopathologic features are distinctive and demonstrate intraepidermal reticular degeneration and cellular necrosis. [2] Most cases remit spontaneously by late adolescence. [3] See the image below.

Characteristic vesicular lesions occur on sun-expo Characteristic vesicular lesions occur on sun-exposed skin and heal with varioliform scarring.


The etiology of hydroa vacciniforme (HV) is uncertain. HV may be a distinct entity distinguished by scarring or may occur within the spectrum of polymorphous light eruption. Skin lesions occur on sun-exposed skin, such as the face, ears, and hands. There is an association between HV occurrence and latent Epstein-Barr virus (EBV) infection, particularly if necrotic lesions are present. [4] T cells positive for EBV-encoded small nuclear RNA (EBER) have been detected in the cutaneous infiltrates of some patients with HV. Ultraviolet-induced cutaneous lesions with histopathology consistent with HV have also been shown to contain EBER-positive cells. [5] In general, the EBV DNA blood load is higher in patients with HV than in patients with other photosensitivity disorders. [6]



The development of hydroa vacciniforme (HV) lesions and their distribution suggest a causal relationship between HV and ultraviolet (UV) exposure, although the pathogenetic mechanism remains unknown. [7] Ultraviolet A (UV-A) radiation is most often implicated, although some older studies suggest ultraviolet B (UV-B) as the causal agent. [8] Two reports of HV in siblings have been documented, suggesting a genetic component to HV. [9] Some cases have occurred in the setting of hematopoietic malignancy. Moreover, HV can be caused by a recent or latent EBV infection. [5]




The frequency of HV varies according to country but is estimated to be around 0.34 cases per 100,000 people.


Males have a higher incidence of HV than females. Males who are affected may also have a longer course of disease than females. [10]


HV predominately affects children aged 3-16 years. [2] In many cases, there is a bimodal age distribution of early childhood (age 1-7 y) and around puberty (age 12-16 y). [8] The mean duration from onset of symptoms to resolution is 9 years. Resolution of symptoms typically occurs in adolescence or young adulthood, although symptoms persist throughout life in some patients. [10] Cases of HV in infants and elderly persons have also been described. [11]


Asians and Latin Americans are more likely to develop severe HV, which includes facial edema, multiple vesicles, and extensive scarring in both sun-exposed and sun-protected skin. [1]



The prognosis is uncertain; classic hydroa vacciniforme (HV) typically remits by adolescence. No mortality is associated with classic HV, but those with lymphoid atypia, rimming of lipocytes, and Amerindian heritage are at significant risk of progression and death. Classic chemotherapy is associated with adverse outcomes. [12]


Patient Education

Patients are advised regarding strict sun avoidance, frequent application of high SPF sunscreens with UV-A blocking agents, and protective clothing.