Track: Case Reports

Abstract:

Hydroa Vacciniforme (HV) is a photosensitive disorder presenting with recurrent photo-distributed papulovesicular eruptions, that subsequently scar. These patients usually have no systemic symptoms, normal hematologic findings, and elevated serum EBV DNA. Severe forms of HV present with widespread eruptions and symptoms like facial edema, fever, lymphadenopathy, ocular and oral lesions, gastrointestinal involvement, and hepatosplenomegaly. Such presentations may suggest an underlying Hydroa Vacciniforme Lymphoproliferative Disorder (HVLPD) requiring systemic therapies, including chemotherapy or stem cell transplantation. We present a 12-year-old male who recently immigrated from Guatemala with a history of eosinophilic esophagitis, presenting with a widespread, rapidly progressing violaceous papulovesicular eruption in the setting of chronic active EBV disease. A biopsy showed superficial necrosis with many EBV-positive T cells within the dermis and subcutis, consistent with HV. Though the patient initially lacked systemic symptoms, the widespread nature of his eruption raised concern for evolving HVLPD. His laboratory workup revealed positive EBV IgG and nuclear antibodies, leukopenia, and thrombocytopenia. He was treated with topical steroids, counseled on sun protection, and discharged. Three weeks later, he re-presented with worsening rash, fever, odynophagia, conjunctivitis, lymphadenopathy, and oral ulcerations. An esophageal biopsy was performed, showing esophagitis with ulceration and EBV positive cells. His laboratory evaluation also revealed pancytopenia and EBV viremia, meeting criteria for HVLPD. The patient ultimately underwent allogeneic peripheral blood stem cell transplantation. This case underscores the importance of early recognition of atypical HV and surveillance for systemic symptoms, which may indicate progression to HVLPD, a potentially life-threatening EBV-driven disorder requiring prompt multidisciplinary care.

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Aisha Jamison