頁籤選單縮合
題 名 | Hydroa Vacciniforme-Like Lymphoma: A Case Report and Literature Review |
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作 者 | Lin, Hung-chien; Chao, Sheau-chiou; Chang, Kong-chao; Lee, J. Yu-yun; | 書刊名 | Dermatologica Sinica |
卷 期 | 28:4 2010.12[民99.12] |
頁 次 | 頁167-172 |
分類號 | 415.692 |
關鍵詞 | Hydroa vacciniforme; Hydroa vacciniforme-like lymphoma; |
語 文 | 英文(English) |
英文摘要 | Hydroa vacciniforme (HV) is a photosensitivity disorder in childhood characterized by recurrent vacciniform vesicles, necrotic ulcers, and scars on sun-exposed areas. HV-like lymphoma is a rare variant of cutaneous T-cell lymphoma. HV, atypical HV and HV-like lymphoma belong to the spectrum of Epstein-Barr virus (EBV)-associated lymphoproliferative disorders. We report a fatal case of HV-like lymphoma in a 31-year-old man with a 16-year history of recurrent vacciniform papulovesicular eruption with crusts and scarring. The rash initially was confined to the sun-exposed areas. Histopathology revealed focal necrosis of the epidermis and subjacent dermis with a superficial lymphocytic infiltrate, consistent with HV. Toward the end of the clinical course, the skin lesions became persistent and spread to nonsun-exposed areas. Repeated biopsies revealed epidermal necrosis with infiltration of CD4+, CD56− lymphocytes in the dermis, some with atypical nuclei, and small blood vessel vasculitis. EBV-encoded RNA (EBER-1)-positive lymphocytes were detected. Progression of his skin lesions was associated with colon ulcers, gingival ulcers, fever, splenomegaly, leukopenia and thrombocytopenia. EBER-1-positive lymphocytes were detected in all biopsy specimens, including the skin, gingiva, and bone marrow; the last also showed infiltrate of atypical lymphocytes with T-cell receptor-γ gene rearrangement. The pathogenic role of UV-irradiation is discussed. |
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