A Rare Case of Hydroa Vacciniforme Lymphoproliferative Disorder in an Adult Male

Summary: Hydroa vacciniforme lymphoproliferative disorder (HV-LPD) is a rare Epstein-Barr virus (EBV)-associated condition predominantly described in pediatric and adolescent populations from South America and Asia. Here, we report a unique case of a previously healthy 26-year-old Guatemalan male with a 6-month history of recurrent erythematous papulonodular lesions associated with burning pain and subjective fevers. Physical examination revealed numerous papulonodules with central hemorrhagic crusting and scattered scars on the face, ears, trunk, and distal extremities, without lymphadenopathy or hepatosplenomegaly. Histopathology demonstrated epidermal necrosis and ulceration with EBER-positive, atypical CD8-positive lymphocytes distributed in a perivascular and periadnexal pattern. Immunohistochemical staining revealed CD56 negativity and positivity for Granzyme B and TIA-1. EBV serology showed elevated nuclear antibody titers, while capsid IgM was negative. Tissue cultures, direct immunofluorescence, and LDH levels were unremarkable. Molecular studies, including T-cell clonality and EBV DNA quantification, are pending. This case highlights a rare presentation of HV-LPD in adulthood, underscoring the importance of clinicopathologic correlation in distinguishing this entity from NK/T-cell lymphoma. Given the variable course and potential progression of HV-LPD to systemic lymphoma, comprehensive work-up and long-term follow-up are critical.