Abstract
Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is a rare Epstein–Barr virus (EBV)-associated lymphoproliferative disease. The disease course of HVLPD varies from an indolent course to progression to aggressive lymphoma. We investigated the characteristics of HVLPD in Korean patients. HVLPD patients at Seoul National University Hospital between 1988 and 2019 were retrospectively analyzed. This study included 26 HVLPD patients who all presented with recurrent papulovesicular and necrotic eruption on the face, neck, and extremities. EBV was detected from the skin tissues of all patients. HVLPD was diagnosed during childhood (age < 18 years) in seven patients (26.9%) and in adulthood (age ≥ 18 years) in 19 cases (73.1%). The median age at diagnosis was 24.0 years (range 7–70 years). HVLPD has various clinical courses, from an indolent course to progression to systemic lymphoma. Fourteen patients (53.8%) developed lymphoma: systemic EBV-positive T-cell lymphoma (n = 9, 34.6%); extranodal natural killer/T-cell lymphoma, nasal type (n = 3, 11.5%); aggressive natural killer/T-cell leukemia (n = 1, 3.8%); and EBV-positive Hodgkin lymphoma (n = 1, 3.8%). Mortality due to HVLPD occurred in five patients (26.3%) in the adult group, while it was one patient (14.3%) in the child group. As lymphoma progression and mortality occur not only in childhood but also in adulthood, adult-onset cases may need more careful monitoring.
Highlights
Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is a rare Epstein–Barr virus (EBV)associated lymphoproliferative disease
According to the revised 4th World Health Organization (WHO) classification, the term HVLPD has been renamed from hydroa vacciniforme-like lymphoma to HVLPD owing to its relationship to chronic active EBV infection (CAEBV) and the broad spectrum of its clinical course1
A total of 26 cases were enrolled with diagnosis of HVLPD based on clinical and histopathologic features between 1988 and 2019 in the Department of Dermatology of Seoul National University Hospital (SNUH)
Summary
Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is a rare Epstein–Barr virus (EBV)associated lymphoproliferative disease. The disease course of HVLPD varies from an indolent course to progression to aggressive lymphoma. HVLPD has various clinical courses, from an indolent course to progression to systemic lymphoma. As lymphoma progression and mortality occur in childhood and in adulthood, adult-onset cases may need more careful monitoring. Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is a rare Epstein–Barr virus (EBV)-associated T-cell or natural killer (NK)-cell lymphoproliferative disease. HVLPD usually affects children, adult and elderly cases have been r eported. HVLPD usually affects children, adult and elderly cases have been r eported2 It is characterized by recurrent erythematous papules and vesicles that leave pitted scars on sun-exposed areas, including the face and forearms. The disease course of HVLPD varies from an indolent course to systemic lymphoma. We report on 26 cases of HVLPD encountered at our hospital during the last three decades
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