Systemic hydroa vacciniforme lymphoproliferative disorder in a patient with chronic active EBV infection

Hydroa vacciniforme lymphoproliferative disorders (HVLPD) fall within the clinical spectrum of chronic active epstein barr virus (EBV) disease (CAEBVD), ranging from localised and/or indolent forms (classic HVLPD) to systemic disease with fever, hepatosplenomegaly and lymphadenopathy (systemic HVLPD). A preadolescent male with 47XYY, multicystic dysplastic kidney, autism spectrum disorder and Attention-deficit/hyperactivity disorder (ADHD) presented with photodistributed non-pruritic, non-painful necrotic papulovesicles accompanied by non-febrile intermittent fatigue and lymphadenopathy. The patient had a history of EBV pneumonia in infancy confirmed by CT scan and was later diagnosed with CAEBV. Despite presenting without the typical risk factors of East Asian or Latin American background, young adulthood onset and long-standing classic HVLPD, the patient's CAEBVD, systemic symptoms and gamma-delta T-cell clonal expansion were consistent with a diagnosis of systemic HVLPD. Systemic HVLPD is high-risk for malignant transformation, requiring close follow-up to evaluate for HV-like T-cell lymphoma. Due to the patient's risk factors and findings consistent with systemic HVLPD, the patient is regularly surveilled for progression to lymphoma.