Hydroa vacciniforme - Skin in Adult
The etiology is unknown but postulated to be immune in nature. The disease has been associated with chronic Epstein-Barr virus (EBV) infection, and EBV-infected cells have been demonstrated in the skin lesions.
The prevalence is estimated to be 0.34 per 100 000 people. In one case series of 17 patients, the mean age at onset was 7.9 years with a range of 1-16 years. Males tend to be affected more severely than females, with a later onset and a longer disease course. The disease is usually sporadic, although rare familial cases have been reported.
Patients typically experience a pruritic or burning sensation within a few hours after exposure to sunlight, followed by the appearance of erythematous macules that progress to papules, vesicles, and necrotic crusts. The lesions are symmetrically located in sun-exposed areas. The rash heals in 1-6 weeks with vacciniforme (poxlike) scarring. In temperate climates, lesions are worse in the spring and summer and improve each winter. Patients are particularly sensitive to ultraviolet radiation A (UVA) light. Rare manifestations include ocular involvement (such as photophobia, conjunctivitis, or keratitis) and ulcerations of the oral mucosa or lip.
Two forms of hydroa vacciniforme have been described:
- Typical hydroa vacciniforme – Localized to sun-exposed areas of the skin and tends to remit in adolescence or young adulthood, although it may persist throughout life. In patients whose disease remits, the mean duration of disease is 9 years.
- Severe hydroa vacciniforme – Characterized by more severe lesions that may be distributed over both sun-exposed and sun-protected areas. During acute episodes, systemic findings are present, including high fever, facial swelling, liver damage, and hematologic abnormalities. An association with hypersensitivity to mosquito bites has been described. Over time, severe hydroa vacciniforme may progress to malignant NK/T-cell lymphoma. In one case series of 11 children, progression to malignancy occurred in 5. Most reports of severe hydroa vacciniforme have occurred in Asia and Latin America.
L56.4 – Polymorphous light eruption
200837006 – Hydroa vacciniforme
- Vesicular polymorphic light eruption (PMLE) – The most common idiopathic photodermatitis, which also presents with a papular eruption on sun-exposed sites, but scarring does not occur.
- Actinic prurigo – This idiopathic photodermatitis mainly affects North, Central, and South American Indians; cheilitis is a distinguishing feature.
- Erythropoietic protoporphyria – Characterized by painful photosensitivity, beginning in childhood, that heals with vermicular (wormlike) scarring, and erythrocyte protoporphyrin levels are markedly elevated.
- Bullous lupus erythematosus (see systemic lupus erythematosus) – Patients typically will have other systemic findings of lupus, positive ANA or anti-double-stranded DNA, and biopsy specimens with characteristic immunofluorescence and histology.