Recent Developments in the Diagnosis and Management of Photosensitive Disorders

Al‐Nahhas, Adil; Oberlin, David; Braunberger, Taylor L.; Lim, Henry W.

doi:10.1007/s40257-018-0365-6

Cited by 21 publications

(29 citation statements)

References 230 publications

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“…This might be as a result of repeated UV exposure reactivating EBV in latent lymphocytes. 14 The emergence of HVLPD is closely associated with ultraviolet light. The malignant transformation of HVLPD has been linked to ultraviolet radiation, and sunlight can both cause and exacerbate skin lesions.…”

Section: Discussionmentioning

confidence: 99%

“… 12 , 13 Previous studies have shown that TCR gene rearrangement is considered a useful tool for distinguishing between classical HV and severe HV. 14 Due to EBV infection, patients may have different levels of EBV DNA in their plasma and Peripheral Blood Mononuclear Cell (PBMC), 15 , 16 and Epstein-Barr virus-encoded RNA (EBER) in situ hybridization of tissues may be positive. In addition, the degree of EBV infection may also be related to the prognosis, but the relevant cases are rarely reported at present.…”

Section: Introductionmentioning

confidence: 99%

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Clinical and Pathological Features of Hydroa Vacciniforme-Like Lymphoproliferative Disorder Along with Risk Factors Indicating Poor Prognosis

Chang¹,

Zhang²,

Lu³

et al. 2023

IDR

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Purpose To examine the clinical and pathological features, laboratory markers, therapeutic options and risk factors indicating poor prognosis of hydroa vacciniforme-like lymphoproliferative disorder (HVLPD). Patients and Methods Seven patients with HVLPD had their clinical and pathological data collected. Immunohistochemical staining, Epstein-Barr virus-encoded RNA (EBER) in situ hybridization experiments, T-cell receptor (TCR) gene rearrangement, RT-PCR tests and the Elisa assay were carried out. Results The main clinical manifestations were papulovesicular lesions and ulcers on the face, neck, or trunk. Five cases had systemic symptoms. Three of the deceased patients had significant facial edema, deep body necrosis, and ulceration. The pathological results demonstrated that lymphocytes infiltrated blood vessels and sweat glands in addition to the dermis and subcutaneous tissues. All patients tested positive for CD3 and EBER. Six cases tested positive for TCRβF1, but none tested positive for TCRδ. TCRγ monoclonal rearrangement, strongly positive expression of TIA-1 and a Ki67 proliferation index of 40% occurred in 3 fatal cases. When compared to the survival group, the plasma EBV DNA in the deceased group was considerably higher ( P <0.05). IFN-γ and TNF-α cytokine levels in patients were higher than in the control group, particularly in the deceased group ( P <0.05). The skin lesions on all patients recovered quickly underwent conservative care. Nonetheless, 3 patients passed away as the disease progressed in its latter stages. Conclusion In our cases, the main infiltrating cells were T cells and the dominant lymphocyte subclass was αβT cells. A significant increase in lgE level, plasma EBV DNA, IFN-γ, and TNF-α cytokine levels, decreased hemoglobin level, strongly positive expression of TIA-1, high Ki67 proliferation index, and positive TCR gene rearrangement are all indicators of a poor prognosis.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Clinical and Pathological Features of Hydroa Vacciniforme-Like Lymphoproliferative Disorder Along with Risk Factors Indicating Poor Prognosis

Chang¹,

Zhang²,

Lu³

et al. 2023

IDR

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“…Since cutaneous lesions of HV patients can be induced by photoprovocation testing, the conceptually accepted hypothesis is that UV radiation with wavelengths between 320 and 390 nm may be the causal agent of HV [8,[25][26][27][28][29]. A single high-dose of UVA irradiation does not induce HV, but repetitive photoprovocation tests at the same site, even with lower doses of UVA irradiation, result in the induction of erythema and vesiculation [29]. However, the mechanism and the chromophore resulting in UV-induced damage in HV are unclear and need further investigation.…”

Section: Uv Irradiation May Induce Hvmentioning

confidence: 99%

“…Monoclonal rearrangement of TRG and/or TRB is regarded as a useful tool for distinguishing classic HV from severe HV [29]. In a large cohort study of HVLPD, 88% of patients carried monoclonal T-cell receptor gene rearrangements [19].…”

Section: Pathological Features and Immunochemical Profilesmentioning

confidence: 99%

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

Chen

Chang

Medeiros

et al. 2020

IJMS

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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.

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Section: Uv Irradiation May Induce Hvmentioning

confidence: 99%

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

Chen¹,

Chang²,

Medeiros³

et al. 2020

Preprint

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Hydroa vacciniforme (HV) is a rare form of photosensitivity disorders 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 NK-cell lymphoproliferations with HV-like cutaneous manifestations, including EBV-positive HV, atypical HV, and HV-like lymphoma. Classic HV occurs in childhood with vesiculopapules 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 therapy 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.

show abstract

Recent Developments in the Diagnosis and Management of Photosensitive Disorders

Cited by 21 publications

References 230 publications

Clinical and Pathological Features of Hydroa Vacciniforme-Like Lymphoproliferative Disorder Along with Risk Factors Indicating Poor Prognosis

Clinical and Pathological Features of Hydroa Vacciniforme-Like Lymphoproliferative Disorder Along with Risk Factors Indicating Poor Prognosis

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

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

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