Epstein-Barr Virus-Associated Vesiculopapular Eruption on the Face of a Patient with Natural Killer T Cell Lymphoma

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.

Marked Increase of Circulating Double-Negative gammadelta T Cells in a Patient With Hydroa Vacciniforme-Like Lymphoma

No abstract available.

Linfoma tipo hidroa vacciniforme en Pediatría / Hidroa vacciniforme type lymphoma in Pediatrics

Se presenta caso de mujer de 14 años que consultó por úlceras orales y lesiones cutáneas de tipo vesicular, papular y algunas con evolución a úlcerocostras de centro necrótico, hemorrágico y de variadas formas y tamaños, distribuidas principalmente en áreas fotoexpuestas como rostro y extremidades superiores e inferiores. La anatomía patológica confirmó linfoma tipo hidroa vacciniforme atípico, considerado primer caso descrito y publicado en Paraguay.

A 14 year old female consulted with oral ulcers and skin lesions like vesicles and papules, some of them are ulcers and crusting with a necrotic and hemorrhagic center, the shape and size of every lesion is different, mainly they are presented in sun-exposed areas like the face, arms and legs. The histopathological finding confirms the atypical hidro vaccinifome-like lymphoma, considered first report and published in Paraguay.

Linfoma de células T símil hidroa vacciniforme / Hydroa vacciniforme-like T cell lymphoma

El linfoma cutáneo primario símil hidroa vacciniforme es una enfermedad poco común decrita principalmente en pacientes pediátricos de origen asiático y latinoamericano. Esta entidad está relacionada con la infección crónica por Virus de Epstein-Barr. Clínicamente se caracteriza por una dermatosis que asemeja a la hidroa vacciniforme con compromiso del estado general y una evolución prolongada con períodos de exacerbación y remisión. Se presenta el caso de una paciente de 6 años de edad que cursa un cuadro de tres años de evolución de lesiones cutáneas en el rostro y las extremidades.

Hydroa vacciniforme-like cutaneous primary lymphoma is a rare disease mainly described in pediatric patients from Asia and Latin-America. This entity is related with chronic Epstein-Barr virus infection and clinically manifests as a hydroa vacciniforme eruption with systemic involvement, it has a long evolution intercalating exacerbations and remissions periods. We present the case of a 6 years old female patient with a three years evolution of skin lesions in face and limbs.

Extensive hydroa vacciniforme / Hidroa vaciniforme extensa

Hydroa Vaciniforme is a very rare photodermatosis that is mainly seen in childhood. An 18 year old female student reported that since the age of 5 she has been suffering necrotic lesions and vesicles lesions in exposed areas, leaving asymptomatic varioliform scars, which worsened in summer. Light microscopy showed epidermal necrosis with lymphocytic infiltration . Sunscreens were prescribed with light improvement.

O Hidroa Vaciniforme é uma fotodermatose muito rara vista geralmente na infância. Uma paciente de 18 anos foi examinada, a qual apresenta lesões vesiculosas e necróticas varioliformes nas áreas fotoexpostas, que evoluem para cicatrizes atróficas, piorando no verão. A microscopia óptica mostrou necrose epidérmica com infiltrado linfocítico. Houve pouca melhora com uso de filtros solares.

Hydroa vacciniforme-like lymphoma: two cases report and literatures review / 中华血液学杂志

<p><b>OBJECTIVE</b>Two children with hydroa vacciniforme-like lymphoma (HVLL) were reported for a better understanding of this disease.</p><p><b>METHODS</b>The clinical manifestation, pathological characteristics, therapeutic outcomes of two children with HVLL were analyzed and presented by compared with what described in literatures.</p><p><b>RESULTS</b>Two 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.</p><p><b>CONCLUSION</b>HVLL 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.</p>

EBV-Positive T/NK-Cell Lymphoproliferative Disease of Childhood

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.

Linfoma tipo hidroa: presentación de un caso / Hydroa-like lymphoma: a case report

El linfoma tipo hidroa es un linfoma cutáneo primario T/NK raro, que viene siendo reportado en países de Asia y Latinoamérica, en la que se ha hallado asociación con infección crónica activa por virus de Epstein-Barr. Se presenta el caso de una paciente de 14 años con un tiempo de enfermedad de tres años, en el que inicialmente presenta eritema y vesiculación de áreas fotoexpuestas de manera episódica y que posteriormente presenta edema facial marcado con zonas violáceas, disminución de peso y falla multiorganica a la que se le hace diagnóstico post mórtem de linfoma tipo hidroa.

Hydroa like-lymphoma is a rare T/NK primary cutaneous lymphoma, which has been reported in people from Asia and Latin-America and has been associated to Epstein-Barr virus (EBV) chronic latent infection. We present the case of a 14 year-old patient with a time since diagnosis of illness of three years. She initially has erythema and vesiculation in sun-exposed areas that appears in a episodic way and then adds overt facial edema and purpuric patches, wasting, weight loss and multiorganic failure. The diagnosis of hydroa-like lymphoma was done post-mortem.

Hidroa vacciniforme / Hydroa vacciniforme

La hidroa vacciniforme (HV) es una fotodermatosis poco frecuente, que usualmente se inicia en la infancia y mejora espontáneamente en la adolescencia. Se caracteriza por la presencia de pápulas, vesículas umbilicadas y costras hemorrágicas en áreas fotoexpuestas, que curan dejando cicatrices varioliformes. La histopatología de las lesiones cutáneas, al igual que la inmunofluorescencia directa, presenta hallazgos característicos pero inespecíficos. El diagnóstico se realiza en base a la correlación clínico-patológica. Recientemente, se ha encontrado una relación entre la HV, el virus de Epstein-Barr y el desarrollo de linfoma. El tratamiento consiste en medidas de fotoprotección, aunque en algunos pacientes se requiere del uso de drogas inmunosupresoras sistémicas

Hydroa vacciniforme (HV) is a rare photodermatosis that usually begins in childhood and improves spontaneously in the adolescence. It is characterized by the presence of papules, umbilicated vesicles and hemorrhagic crusts that heal with vacciniform scarring in photoexposed areas. The histopathology of the skin lesions, as well as the direct immunofluorescence findings, is characteristic but nonspecific. The diagnosis is achieved based on clinico-pathological correlation. A relationship between the HV, Epstein-Barr virus and the development of lymphoma has been recently found. The treatment consists of photoprotective measures, but some cases require the use of systemic immunosuppressive drugs

Virus de Epstein-Barr y piel / Epstein-Barr virus and skin

La infección por el virus de Epstein-Barr puede afectar de manera muy variada al ser humano en sus diferentes etapas de la vida. Algunas veces las manifestaciones son asintomáticas o con síntomas poco específicos y otras puede presentarse como una enfermedad de severidad y duración variable, que puede desarrollar un proceso maligno. Hasta el momento se ha estudiado muy poco sobre la relación del virus de Epstein-Barr y piel, por lo que se trata de hacer una revisión de esta enfermedad y sus diferentes expresiones en la piel.

Epstein-Barr virus infection can occur in different manners, affecting humans throughthe different stages of life. Sometimes, manifestations are asymptomatic or non specific;conversely it can occur as a disease of variable severity and duration, and occasionallyeventuate into malignancy. So far little has been studied on the relationship betweenEpstein-Barr virus and skin; therefore we present this review of the disease and its various expressions upon the skin.

Epstein-Barr Virus Associated Hydroa Vacciniforme-like Eruption / 대한피부과학회지

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.

A Case of Hydroa Vacciniforme

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.

Adult Case of Hydroa Vacciniforme

Hydroa vacciniforme [HV] is a rare acquired photodermatosis, usually with onset in childhood and characterized by vesicles, crust and scar formation that follow exposure to sunlight.Vacciniform scars of face and dorsa of the hands are common features but oral ulcer and eye signs also rarely occur. It usually resolves before adult age. A rare manifestation of the disease would be persistence until adult age which is presented in this report

Linfoma CutáneoT/NK tipo nasal símil hidroa vacciniforme / Hydroa-like cutaneous T/NK-cell lymphoma, nasal type

El linfoma T/NK cutáneo símil hidroa vacciniforme (HV) es un linfoma poco común que afecta principalmente a niños asiáticos e indoamericanos. Se inicia con edema, vesículas, costras y cicatrices de tipo vacciniforme. De evolución prolongada, tiende a agravarse con los años, desfigurando y comprometiendo física y psíquicamente al paciente. Debido a la detección tardía del linfoma, en general el pronóstico de vida de estos pacientes es malo. Se presenta una paciente de 20 años con linfoma NK de tipo nasal, que comenzó a los 13 años con un cuadro de HV de mala evolución. Los autores proponen que en pacientes con HV atípica un tratamiento en etapas tempranas de la enfermedad podría mejorar su pronóstico.

The hydroa-like cutaneous T-cell lymphoma is not a very common lymphoma,children from Asia and indoamericans are mainly aff ected. Theybegin with edema, vesicles, crusts and vacciniform scars with a prolongedevolution increasing through to the years. This illness aff ects the patientsphysically and psychologically because of its disfi guring eff ects.Due to the late detection of the lymphoma, generally the prognosis ofthese patients is poor. A patient of 20 years old, who suff ered of NK-celllymphoma nasal type, is presented. She began with hydroa vacciniformeat the age 13 years old, she developed a T/NK lymphoma and died. Theauthors propose that on patients with atypical hydroa vacciniformes, atreatment in earlier stages could off er a better prognosis (Dermatol Argent2009;15(5):350-353).

Hydroa vacciniforme-like cutaneous T cell lymphoma: a case report and literature review / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the clinical features, diagnosis and therapy of hydroa vacciniforme-like cutaneous T cell lymphoma.</p><p><b>METHODS</b>The 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.</p><p><b>RESULTS</b>The 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.</p><p><b>CONCLUSIONS</b>Hydroa 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.</p>

A Case of Epstein-Barr Virus-associated Hydroa Vacciniforme

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.

Linfoma cutáneo de células T tipo hidroa / Cutaneous T-cell lymphoma type hydroa

El linfoma cutáneo tipo hidroa es un tipo de linfoma relacionado al virus Epstein Barr que afecta a niños y jóvenes, y se caracteriza por lesiones que semejan al hidroa vacciniforme. Se presenta el caso de una paciente adulta sin antecedentes patológicos con compromiso cutáneo facial.

The cutaneous T-cell lymphoma type hydroa is a type of lymphoma related to Epstein Barr virus that affects children and young people, and characterized by lesions that resemble the hydroa vacciniforme. A case of a female adult patient without pathological history and with skin lesions in face.

Hydroa vacciniforme in an adolescent girl

Hydroa vacciniforme [HV] is a rare photosensitivity disorder with onset in childhood. Recurrent vesicles, bullae and crusting occur on sun-exposed skin that heals with vacciniform scarring. This rare disorder is hereby reported in an adolescent girl who developed a severe episode of hydroa vacciniforme on a hot sunny day during harvesting season. She had repeated episodes afterwards in succession until she was appropriately sun-screened. Her lesions healed without scarring because of early reporting, short-lived episodes, early diagnosis and response to appropriate therapy