Back issues No.4 - 2010 / Case Report  

Full Text PDF


Hydroa vacciniforme-like lymphoma: a case report and literature review
Hung-Chien Lin, Sheau-Chiou Chao, Kong-Chao Chang, J. Yu-Yun Lee
DERMATOLOGICA SINICA 28 (2010) 167–172

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.

You are visitors    
Copyright © 2008 THE Taiwanese Dermatological Association. All Rights Reserved. Email: Tel:886-2-2518-5126~7 Fax:886-2-2518-5128