Hydroa vacciniforme-like lymphoma: A case report and literature review

Hung Chien Lin, Sheau Chiou Chao, Kong Chao Chang, J. Yu Yun Lee

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4 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)167-172
Number of pages6
JournalDermatologica Sinica
Volume28
Issue number4
DOIs
Publication statusPublished - 2010

All Science Journal Classification (ASJC) codes

  • Dermatology

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