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      Epstein-Barr Virus-Associated Vesiculopapular Eruption on the Face of a Patient with Natural Killer T Cell Lymphoma

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          Abstract

          Unlike typical hydroa vacciniforme (HV), Epstein-Barr virus (EBV)-associated HV-like eruption is more variable in its clinical manifestations. In some patients, progression to lymphoma or leukemia has been reported, which are characterized by the T-cell immunophenotype. Here, we report the first Korean case of EBV-associated vesiculopapular eruption on the face of a patient with natural killer (NK)/T cell lymphoma. A 32-year-old Korean man presented with a late adolescent-onset recurrent necrotic papulovesicles on his face. The patient was previously diagnosed with EBV-associated NK/T cell lymphoma of the oral cavity and also had childhood-onset hypersensitivity to mosquito bites. Biopsy of his facial skin showed EBV-associated vesiculopapular eruptions, though ultraviolet provocation did not reproduce the skin lesions. EBV viral load in his peripheral blood was detected but low. The patient was treated with systemic chemotherapy. The lymphoma went into remission, but the facial EBV-associated vesiculopapular eruption had a relapsing and remitting course.

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          Pathogenic link between hydroa vacciniforme and Epstein-Barr virus-associated hematologic disorders.

          To determine the pathogenic association of latent Epstein-Barr virus (EBV) infections with both typical hydroa vacciniforme (HV) and severe HV-like eruptions, and to survey the complications and outcomes of patients.
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            Hypersensitivity to mosquito bites as the primary clinical manifestation of a juvenile type of Epstein-Barr virus-associated natural killer cell leukemia/lymphoma.

            Hypersensitivity to mosquito bites or mosquito allergy is a mysterious disorder that has been reported mainly in Japanese patients (at least 58 patients) in the first two decades of life. The skin lesion at bite sites is typically a bulla that develops into necrosis. Patients simultaneously exhibit a high temperature and general malaise and subsequently may experience lymphadenopathy and hepatosplenomegaly. Recent studies have revealed that this mosquito hypersensitivity is associated with chronic Epstein-Barr virus infection and natural killer cell leukemia/lymphoma. The natural killer cell, infected with monoclonal (or oligoclonal) Epstein-Barr virus, seems to be involved in the pathogenesis of the hypersensitivity. Half of the patients reported died of hemophagocytic syndrome (or malignant histiocytosis), granular lymphocyte proliferative disorder, or lymphomas. We propose that this disease, defined as the triad of hypersensitivity to mosquito bites, chronic Epstein-Barr virus infection, and natural killer cell leukemia/lymphoma, is a clinical entity mostly seen in Asians.
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              Epstein-Barr virus-associated peripheral T-cell lymphoma in adults with hydroa vacciniforme-like lesions.

              We describe two Korean adult patients who had necrotizing papulovesicles mainly on their faces. Skin biopsy specimens showed perivascular and periadnexal infiltrate of atypical lymphoid cells with vasculitis in the dermis and subcutaneous tissue. In situ hybridization demonstrated a latent infection of Epstein-Barr virus in the majority of lymphoid cells in the dermis. These patients were diagnosed as having T-cell lymphoma. Interestingly, large granular lymphocytosis was found in the peripheral blood of Case 2.
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                Author and article information

                Journal
                Ann Dermatol
                Ann Dermatol
                AD
                Annals of Dermatology
                The Korean Dermatological Association; The Korean Society for Investigative Dermatology
                1013-9087
                2005-3894
                October 2017
                25 August 2017
                : 29
                : 5
                : 618-620
                Affiliations
                Department of Dermatology, Seoul National University Hospital, Seoul, Korea.
                [1 ]Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.
                Author notes
                Corresponding author: Kwang Hyun Cho, Department of Dermatology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul 03080, Korea. Tel: 82-2-2072-2417, Fax: 82-2-742-7344, khcho@ 123456snu.ac.kr
                Article
                10.5021/ad.2017.29.5.618
                5597657
                5a895210-9a0a-4626-8d40-dce88e7cc32d
                Copyright © 2017 The Korean Dermatological Association and The Korean Society for Investigative Dermatology

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 22 August 2016
                : 08 November 2016
                : 11 November 2016
                Categories
                Case Report

                Dermatology
                epstein-barr virus,epstein-barr virus-associated hydroa vacciniforme-like eruption,epstein-barr virus-associated vesiculopapular eruption,hydroa vacciniforme,natural killer t cell lymphoma

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