Cases reported "Hydroa Vacciniforme"

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1/4. 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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keywords = dermis
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2/4. hydroa vacciniforme-like eruptions in a patient with chronic active EB virus infection.

    We report a case of chronic active Epstein-Barr (EB) virus infection (CAEBV) associated with skin eruptions mimicking hydroa vacciniforme (HV) in a 4-year-old boy. The patient had repeated episodes of vesiculo-necrotic eruptions on the face, scalp, and bilateral forearms one year before the first visit to our department. General symptoms including fever, hepatosplenomegaly, abnormal liver function, and cervical lymph node swelling were noted three months before the first visit. At the first visit, small, bean-sized, erythemic papules with central necrosis were observed on the face and anterior chest wall. thumb-sized ulcers with crust were present on the bilateral forearms. Histopathological examination of an erythematous lesion in the submandibular area revealed parakeratosis with a thick crust, mild spongiosis in the epidermis, and a dense infiltration of lymphoid cells into the dermis and perivascular space. Laboratory examination showed EBNA x 40, EBV VCA IgG x 1,280, and EBV dna (PCR) 8 x 10(4). EBV-encoded small nuclear rna (EBER) positive cells were detected in the dermis by an in situ hybridization (ISH) method. Large granular lymphocytes (65%) with the NK cell phenotype were found in the peripheral blood. A real time PCR method showed 171,741 copies/ micro g dna in CD 16 positive cells. Although latent EBV infection-associated eruptions have been documented, detailed skin manifestations in CAEBV are less well known.
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keywords = dermis
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3/4. Artificial reproduction of atypical hydroa vacciniforme caused by latent EpStein-Barr virus infection.

    BACKGROUND: Cases of hydroa vacciniforme-like eruptions caused by latent Epstein-Barr virus (EBV) infection have been reported in asia and mexico, some of which progressed to lymphoproliferative disorders. The precise clinical features of the disease are not, however, clear. methods: We performed an artificial provocation test with repeated exposures of ultraviolet A (UVA) and UVB in an 8-year-old Korean girl who had severe hydroa vacciniforme-like skin eruptions on exposed areas. RESULTS: The patient showed features of latent EBV infection serologically, and an in situ hybridization study indicated that most infiltrating mononuclear cells in the dermis were positive for EBV. After three exposures to UVA irradiation with a total dose of 90 J/cm2, erythema and vesicles appeared on the UVA-irradiated site but not on the UVB-irradiated site. CONCLUSIONS: Atypical hydroa vacciniforme caused by latent EBV infection could be reproduced by repeated UVA irradiation. Further study on the photo-protection would be necessary to prevent its progression to lymphoproliferative malignancies.
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keywords = dermis
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4/4. Coexistence of hydroa vacciniforme and malignant lymphoma.

    Ten years after the onset of hydroa vacciniforme (HV), a 16-year-old boy developed edema and red induration of the face, ears, and dorsa of the hands. Aggravation of cutaneous manifestations was associated with general malaise, headache, fever, lymphadenopathy, hepatosplenomegaly, and an increase of several serum enzymes. The vesicle was situated intraepidermally with thrombosis and hemorrhage underneath. This confirmed the diagnosis of HV. In addition, dense cell infiltrate was seen in deep dermis and subcutaneous tissue. Histologic and immunohistochemical studies of the dermal cell infiltrate and lymph node showed an infiltrate of helper T lymphocytes with an atypia and histiocytic cells (S100[-], alpha-subunit[ ]). Hence, we concluded HV and malignant lymphoma coexisted in this patient.
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keywords = dermis
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