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Hydroa vacciniforme is a rare photodermatosis described by Bazin in the 19th century. It is an acquired condition that begins in childhood and resolves spontaneously, often in adolescence.
First symptoms usually occur before the age of ten. Boys are preponderantly affected. The symptoms are characteristic of the condition. After significant sun exposure in summer, vesicles appear within 24 hours on the face, forearms and backs of hands. A burning sensation and slight fever may accompany the attacks. Then the lesions become confluent and crusted. Within a few weeks, crusts detch to leave depressed pocklike scars that are described as “varioliform”. The disease course is marked by symptoms recurring each time patients are exposed to the sun. However, attacks become less severe and disappear at puberty in the majority of cases. Long-term follow-up is required because it is possible that the patient develop lymphoma (cancer of lymphatic system).
Little is known about the cause of this disease. The role played by sunlight exposure related to UVA in lesions triggering is mentioned. Recently, a possible role of Epstein-Barr virus (EBV) has been suggested in hydroa vacciniforme.
Sunscreens should be used during sunny seasons (spring and summer). This is effective as long as sufficient quantities are applied regularly. A broad-spectrum sunscreen (blocking both UVA and UVB rays) with high protection factor of 50+ is recommended. In view of the potential for the eyes to be affected, protective sunglasses is also indicated. It is recommended to wear protective clothing (garments offering full coverage and a hat).
Phototherapy could be prescribed when protection measures fail. It consists of undergoing medically prescribed sessions of UV exposure (PUVA or narrow band UVB) which take place either in a dermatology clinic or hospital. Several sessions are carried out to begin in early spring so that the skin achieves the best possible tolerance for sunlight.
Other medications as beta-carotene, antimalarials, diet fish oil and thalidomide) have been used with variable efficacy. More recently, anti-viral drugs as aciclovir and valaciclovir have been tried, in view of Epstein-Barr virus being connected to hydroa vacciniforme, with good results.
Bazin’s Hydroa vacciniforme.
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