Hydroa vacciniforme is a very rare photosensitivity disorder of childhood that is characterized by pruritic or painful vesicles in photo-distributed areas. These lesions usually follow sunlight exposure and notably heal with permanent varioliform-type scarring. Hydroa vacciniforme was originally described as a benign disorder in western countries due to the absence of systemic symptoms and spontaneous regression of disease in adolescence or young adulthood. However, a more severe and disfiguring form has appeared in children from Latin America and Asia, with the name hydroa vacciniforme-like lymphoma. Hydroa vacciniforme-like lymphoma has been linked to Epstein-Barr virus (EBV) infection and is currently recognized by the World Health Organization classification of lymphomas as a subgroup of EBV+ T-cell lymphoproliferative disorders of childhood.
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