Hydroa vacciniforme-like Epstein-Barr virus-associated monoclonal T-lymphoproliferative disorder in a child.
Hydroa vacciniforme (HV) is a chronic photosensitivity disorder induced by ultraviolet radiation. Hydroa vacciniforme-like lymphoma is a rare cutaneous T-cell lymphoma occurring mainly in childhood. Recent studies have demonstrated an association between chronic latent Epstein-Barr virus (EBV) infection and both the benign skin disorder and the lymphoma. The authors report a 6-year-old boy with chronic EBV infection, HV-like skin eruptions, and chronic hepatitis. Histopathologic examination of a skin biopsy specimen demonstrated epidermal ballooning degeneration and dense superficial and deep perivascular and periappendageal lymphoid cell infiltrates extending to the fat lobules. Some blood vessels in the deep plexus were infiltrated by predominantly CD4+ and TIA-1+ cytotoxic T cells. The EBV genomes were found within tissue from three skin biopsies and peripheral blood cells. Monoclonal T-cell receptor gene rearrangement was present in skin biopsy specimens. Although no lymphoma has been found during 2 years of follow-up treatment, the possibility of lymphoma developing out of the current smoldering stage is of concern. The clinical manifestations of lymphoproliferative disorder and chronic active EBV infection are discussed.
Wu
Yu-Hung
YH
Department of Dermatology, Mackay Memorial Hospital, and Mackay Medicine, Nursing and Management College, Taipei, Taiwan.
Chen
Hsiu-Chin
HC
Hsiao
Pa-Fan
PF
Tu
Mei-Ing
MI
Lin
Yang-Chih
YC
Wang
Tao-Yuan
TY
D015703:Antigens, CD / Q000378:metabolism; D002648:Child; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D004854:Herpesvirus 4, Human / Q000302:isolation & purification*; D006801:Humans; D006837:Hydroa Vacciniforme / Q000276:immunology / Q000473:pathology / Q000821:virology*; D008232:Lymphoproliferative Disorders / Q000276:immunology / Q000473:pathology / Q000821:virology*; D008297:Male; D016133:Polymerase Chain Reaction; D012867:Skin / Q000473:pathology / Q000821:virology; D013601:T-Lymphocytes / Q000276:immunology
D015703:Antigens, CD