Hydroa vacciniforme‐like Epstein‐Barr virus‐associated monoclonal T‐lymphoproliferative disorder in a child

Abstract

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.

Hydroa vacciniforme-like lymphoma: a chronic EBV+ lymphoproliferative disorder with risk to develop a systemic lymphoma

“…4,16 CD30 expression was found in a portion of the reported cases in the literature. 3 Since the incorporation of HVLL into the WHO lymphoma classification, several controversial issues have been raised that remain to be clarified.…”

“…9,[13][14][15] In contrast, only rare cases of CD4 1 T-cell phenotype have been described. 16,17 The lymphoid cells, regardless of cell-type derivation, are positive for cytotoxic markers such as granzyme B and T-cell intracellular The online version of this article contains a data supplement.…”

Chronic Active Epstein-Barr Virus Infection-Associated Hydroa Vacciniforme-Like Eruption and Behçet's-Like Orogenital Ulcers

“…HV-like eruption is a representative cutaneous manifestation of EBV-associated lymphoproliferative disorder and is reported mainly in Asia [4,13,14,15,16,17] and Latin America [18,19]. HV-like eruption has similar clinical and pathologic findings to those of HV.…”

“…Of particular concern is the prognosis that HV-like eruption occasionally progresses to hematologic malignancies, whereas typical HV is an almost entirely self-limited disease. Recently, HV and HV-like eruption were considered variants within the same disease spectrum of EBV-associated lymphoproliferative disorders caused by a cytotoxic T cell response against virus-infected cells, and not distinct diseases [13,15,20]. Our patient was diagnosed with chronic active EBV infection with a high peripheral blood viral load.…”

Atypical Hydroa Vacciniforme-Like Epstein-Barr Virus Associated T/NK-Cell Lymphoproliferative Disorder

“…EBER-positive cells are detected in the infiltrated lymphocytes of classic HV and atypical HV-like eruptions. 10,[15][16][17] It has been suggested that classic HV and atypical HV-like eruptions are variants within the same disease spectrum of EBV-T/NK-LPD. 10,15 In our case, the second skin biopsy demonstrated typical findings of HV.…”