Unlike typical hydroa vacciniforme (HV), Epstein-Barr virus (EBV)-associated HV-like eruption is more variable in its clinical manifestations. In some patients, progression to lymphoma or leukemia has been reported, which are characterized by the T-cell immunophenotype. Here, we report the first Korean case of EBV-associated vesiculopapular eruption on the face of a patient with natural killer (NK)/T cell lymphoma. A 32-year-old Korean man presented with a late adolescent-onset recurrent necrotic papulovesicles on his face. The patient was previously diagnosed with EBV-associated NK/T cell lymphoma of the oral cavity and also had childhood-onset hypersensitivity to mosquito bites. Biopsy of his facial skin showed EBV-associated vesiculopapular eruptions, though ultraviolet provocation did not reproduce the skin lesions. EBV viral load in his peripheral blood was detected but low. The patient was treated with systemic chemotherapy. The lymphoma went into remission, but the facial EBV-associated vesiculopapular eruption had a relapsing and remitting course.
Adult ; Biopsy ; Culicidae ; Drug Therapy ; Herpesvirus 4, Human ; Humans ; Hydroa Vacciniforme ; Hypersensitivity ; Leukemia ; Lymphoma* ; Mouth ; Natural Killer T-Cells* ; Skin ; T-Lymphocytes ; Viral Load

No abstract available.
Child, Preschool ; Female ; Flow Cytometry ; Humans ; Hydroa Vacciniforme/*diagnosis/pathology ; Immunophenotyping ; Lymphocytosis/complications ; Lymphoma/*diagnosis ; Receptors, Antigen, T-Cell, gamma-delta/genetics/*metabolism ; STAT3 Transcription Factor/genetics/metabolism ; Sequence Analysis, DNA ; Skin/metabolism ; T-Lymphocytes/*metabolism

OBJECTIVETwo children with hydroa vacciniforme-like lymphoma (HVLL) were reported for a better understanding of this disease.

METHODSThe clinical manifestation, pathological characteristics, therapeutic outcomes of two children with HVLL were analyzed and presented by compared with what described in literatures.

RESULTSTwo children were girls, who treated firstly in the hospital in May 2012, July 2012 and their duration were 1 years, more than 10 years respectively. Their clinical manifestations were both limbs and craniofacial polymorphous rashes. Pathological findings revealed that the dermis and subcutaneous tissue were profiled by atypical lymphocytic infiltration. Immunohistochemistry showed that the infiltration of cells from T/NK cell, and Epstein-Barr virus encoded small RNA (EBER)(+). Case 1 was treated with chemotherapy, but her condition continued to deteriorate. Case 2 just received symptomatic treatment, her skin lesions gradually reduced and rash disappeared completely 2 months later.

CONCLUSIONHVLL is found with special clinical manifestation, its diagnosis mainly depend on skin biopsy and immunohistochemistry, there is no specific treatment method now, and its prognosis still needs further research.

Child ; Child, Preschool ; Female ; Humans ; Hydroa Vacciniforme ; Lymphoma, T-Cell, Cutaneous ; Skin Neoplasms

BACKGROUND: Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH), EBV-positive systemic T-cell lymphoproliferative disease (STLPD) of childhood, and chronic active EBV (CAEBV) infection may develop after primary EBV infection. This study reviewed the clinicopathological spectrum of EBV-associated T- and natural killer (NK)-cell LPD, including STLPD and CAEBV infection, with an analysis of T-cell clonality. METHODS: Clinicopathological features of seven patients with EBV-associated HLH or STLPD and 12 patients with CAEBV infection were reviewed. Immunohistochemical staining and a T-cell receptor (TCR) gene rearrangement study were performed. RESULTS: STLPD and EBV-positive HLH showed significantly overlapping clinicopathological findings. One patient with STLPD and one patient with EBV-positive HLH demonstrated moderate to severe atypia of the infiltrating lymphocytes, whereas the remaining patients lacked significant atypia. Twelve patients had CAEBV infection, four of whom suffered mosquito-bite hypersensitivity, five showed NK lymphocytosis, and one suffered hydroa vacciniforme. Infiltrating lymphocytes were predominantly small and devoid of atypia. Hemophagocytic histiocytosis was found in seven of 11 patients. Monoclonality was detected in three (50%) of the six patients with successful TCR gene analysis. CONCLUSIONS: EBV-positive HLH and STLPD share similar clinicopathological findings and may constitute a continuous spectrum of acute EBV-associated T- or NK-cell proliferative disorders. The distinction of EBV-positive T-cell LPD from EBV-positive HLH may be difficult during routine diagnoses because of the technical limitations of clonality assessment.
Epstein-Barr Virus Infections ; Gene Rearrangement ; Genes, T-Cell Receptor ; Herpesvirus 4, Human ; Histiocytosis ; Humans ; Hydroa Vacciniforme ; Hypersensitivity ; Killer Cells, Natural ; Lymphocytes ; Lymphocytosis ; Lymphohistiocytosis, Hemophagocytic ; Lymphoma, T-Cell ; Lymphoproliferative Disorders ; Receptors, Antigen, T-Cell ; T-Lymphocytes

Adolescent ; Adult ; Female ; Humans ; Hydroa Vacciniforme ; Lymphoma, T-Cell, Cutaneous ; Male ; Skin Neoplasms

Child ; Female ; Humans ; Hydroa Vacciniforme ; Hypersensitivity ; Insect Bites and Stings

Hydroa vacciniforme (HV) is a rare self-limiting disease characterized by vesicles and crust formation after sun exposure. However, cases with HV-like eruptions and severe systemic symptoms have also been described. It had been regarded as a different entity from typical HV. But latent Epstein-Barr Virus (EBV) infection has also been detected in typical HV patients and the possibility has been suggested that typical HV and atypical HV are variants within the same disease spectrum of EBV-associated lymphoproliferative disorders, which ranges from typical HV as a benign pole to severe HV progressing to hematologic malignancy as a malignant pole. Chronic active Epstein-Barr virus infection (CAEBV) is an atypical illness associated with serologic evidence of persistent EBV infection. The extremely high levels of EBV measured in affected tissue or peripheral blood are the most recently proposed diagnostic criteria. Recently, we found high levels of EBV in the peripheral blood of patients with EBV-associated HV-like eruptions.
Epstein-Barr Virus Infections ; Hematologic Neoplasms ; Herpesvirus 4, Human ; Humans ; Hydroa Vacciniforme ; Lymphoproliferative Disorders ; Solar System

Hydroa vacciniforme (HV) is a rare and chronic pediatric disorder that is characterized by photosensitivity and recurrent vesicles that heal with vacciniforme scarring. The pathogenesis of HV is unknown; no chromosome abnormality has been identified. HV patients have no abnormal laboratory results, so the diagnosis of HV is based on identifying the associated histological findings in a biopsy specimen and using repetitive ultraviolet phototesting to reproduce the characteristic vesicles on a patient's skin. Herein, we present a case of HV in a 7-year-old female who was diagnosed with HV according to histopathology and ultraviolet phototesting.
Biopsy ; Child ; Chromosome Aberrations ; Cicatrix ; Female ; Humans ; Hydroa Vacciniforme ; Skin

OBJECTIVETo study the clinical features, diagnosis and therapy of hydroa vacciniforme-like cutaneous T cell lymphoma.

METHODSThe clinical presentations and the findings of laboratory examinations and skin biopsy of affected tissue in a child with hydroa vacciniforme-like cutaneous T cell lymphoma were retrospectively reviewed.

RESULTSThe child manifested as rash, fever and lymph node intumesce. Rash was pantomorphia, including edematous erythema, vesicles, crusts, necrosis and depressed scar, and it was mild in winter and severe in summer, mainly involving in the face and extremities. Epstein-Barre virus (EBV)-IgM was positive. Histopathological findings revealed focal lymphocyte invasion in subcutaneous panniculus adiposus, mainly surrounding the blood vessels. Immunohistochemistry showed CD3 (+), CD43 (+), CD20 (-), pax-5 (-), TIA (+), CD5 (+), CD8 (+), Granmye (+) and CD4 (-). The clinical symptoms were improved after glucocorticoid treatment in this child.

CONCLUSIONSHydroa vacciniforme-like cutaneous T cell lymphoma has special clinical manifestations. This disorder may be definitely diagnosed by skin biopsy of affected tissue and immunohistochemistry assay. Glucocorticoid treatment is effective. EBV infection may be related to the development of this disorder.

Child, Preschool ; Female ; Humans ; Hydroa Vacciniforme ; pathology ; Lymphoma, T-Cell, Cutaneous ; drug therapy ; immunology ; pathology ; Skin ; pathology ; Skin Neoplasms ; drug therapy ; immunology ; pathology

Hydroa vacciniforme (HV) is a photosensitivity disorder characterized by recurrent necrotic vesiculopapules on sun-exposed areas, which heal spontaneously during adolescence. Recently, an association has been reported between latent Epstein-Barr virus (EBV) infection and atypical HV-like eruption and malignant potential. However, latent EBV infection has also been reported in the setting of typical HV. An 11-year-old girl presented with recurrent, scattered, discrete vesicular eruptions with scarring on the face and the extensor surfaces of both forearms. In-situ hybridization was carried out to detect latent EBV infection. Based on the clinical and histopathological findings, typical EBV-associated HV was suspected.
Adolescent ; Child ; Chimera ; Cicatrix ; Epstein-Barr Virus Infections ; Forearm ; Herpesvirus 4, Human ; Humans ; Hydroa Vacciniforme ; Photosensitivity Disorders