Hydroa Vacciniforme and Hydroa Vacciniforme-Like Lymphoproliferative Disorder: A Spectrum of Disease Phenotypes Associated with Ultraviolet Irradiation and Chronic Epstein–Barr Virus Infection

Abstract

Hydroa vacciniforme (HV) is a rare form of photosensitivity disorder in children and is frequently associated with Epstein–Barr virus (EBV) infection, whereas HV-like lymphoproliferative disorders (HVLPD) describe a spectrum of EBV-associated T-cell or natural killer (NK)-cell lymphoproliferations with HV-like cutaneous manifestations, including EBV-positive HV, atypical HV, and HV-like lymphoma. Classic HV occurs in childhood with papulovesicules on sun-exposed areas, which is usually induced by sunlight and ultraviolet irradiation, and mostly resolves by early adult life. Unlike classic HV, atypical or severe HV manifests itself as recurrent papulovesicular eruptions in sun-exposed and sun-protected areas associated occasionally with facial edema, fever, lymphadenopathy, oculomucosal lesions, gastrointestinal involvement, and hepatosplenomegaly. Notably, atypical or severe HV may progress to EBV-associated systemic T-cell or natural killer (NK)-cell lymphoma after a chronic course. Although rare in the United States and Europe, atypical or severe HV and HV-like lymphoma are predominantly reported in children from Asia and Latin America with high EBV DNA levels, low numbers of NK cells, and T cell clones in the blood. In comparison with the conservative treatment used for patients with classic HV, systemic therapy such as immunomodulatory agents is recommended as the first-line therapy for patients with atypical or severe HV. This review aims to provide an integrated overview of current evidence and knowledge of HV and HVLPD to elucidate the pathophysiology, practical issues, environmental factors, and the impact of EBV infection.

Correlations Between Intestinal Microbial Community and Hematological Profile in Native Tibetans and Han Immigrants

“…Phascolarctobacterium is a producer of SCFAs, including acetate and propionate. The gut microbiota sustains hematopoiesis ( Chen et al, 2020 ). The harvesting of nutrients from the diet is a well-recognized feature of the symbiotic relationship between the microbiota and its host throughout evolution ( Baeckhed et al, 2005 ).…”

“…Phascolarctobacterium is a producer of SCFAs, including acetate and propionate. The gut microbiota sustains hematopoiesis (Chen et al, 2020).…”

Chronic active EB virus infection with hemophagocytic lymphohistiocytosis and progression to aggressive NK cell leukemia: a case report and review of the literature

“…It shows a broad spectrum of clinical manifestations, from localized to more systemic diseases. The Local symptoms are mainly cutaneous lesions, such as hydroa cacciniforme-like lymphoproliferative disorder and severe mosquito bite allergy (2). The systemic CAEBV-T/NK is characterized by fever, hepatosplenomegaly, and lymphadenopathy, with or without any cutaneous manifestations (1,3).…”

Hydroa vacciniforme‐like lymphoproliferative disorder with eye involvement

“…1 It is characterized by recurrent vesiculopapular eruptions on sun-exposed and sun-protected skin, especially photo-exposed areas. 2 Some patients progress to systemic syndromes including fever, malaise, lymphadenopathy, and hepatosplenomegaly. Oculomucosal lesions were observed in six (26%) of 23 patients with HVLPD in one study.…”

“…Hydroa vacciniforme‐like lymphoproliferative disorder (HVLPD) is a spectrum of T‐cell or natural killer (NK)‐cell lymphoproliferation associated with Epstein‐Barr virus (EBV) infection 1 . It is characterized by recurrent vesiculopapular eruptions on sun‐exposed and sun‐protected skin, especially photo‐exposed areas 2 . Some patients progress to systemic syndromes including fever, malaise, lymphadenopathy, and hepatosplenomegaly.…”