Hydroa vacciniforme-like lymphoproliferative disorder in Ecuadorian children: A case series.

We report the clinical and histopathological features of hydroa vacciniforme-like lymphoproliferative disorder in five indigenous and Mestizo children. All the children resided at higher altitudes, experiencing maximal solar exposure. All cases presented with prurigo along with Epstein-Barr virus infection. Histopathologic examination showed an atypical, CD30 + lymphocytic infiltrate with angiocentricity in all, while three cases demonstrated panniculitis-like infiltrate.

Oral manifestations of Hydroa vacciniforme-like lymphoproliferative disorder: a clinicopathological study of a Peruvian population.

BACKGROUND: Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is a chronic Epstein-Barr virus (EBV)-positive lymphoproliferative disease which may either present as an indolent condition or progress to a systemic T-cell lymphoma. METHODS: All HVLPD diagnosed over a 10-year period were retrieved, and clinical data regarding sex, age, oral and systemic manifestations, and clinical follow-up were obtained. Immunohistochemistry was done in order to characterize the lymphoid cells, and in situ hybridization was used to demonstrate the presence of EBV. RESULTS: Eleven cases were included, with a male predominance and a mean age of 25.1 years. Buccal mucosa and the lips were the most affected oral sites, appearing as painful ulcers. All patients exhibited facial oedema, usually affecting the lips, nose and periorbital region. The clinical course was gradual but progressive, with four patients having fever and 3 showing lymphadenopathies. All cases showed a moderate to severe lymphocytic infiltrate with angiotropism, angiocentricity and epidermotropism. Two cases affecting the lip skin exhibited a periappendageal lymphocytic infiltrate. Few large pleomorphic cells were found, surrounded by smaller and medium-sized lymphoid cells, as well as reactive plasma cells, macrophages, neutrophils and eosinophils. All lesions exhibited a cytotoxic T-cell (CD8+) phenotype with a variable proliferative index. All cases were associated with EBV, and all patients died due to complications of the disease. CONCLUSIONS: HVLPD is a rare disease that may show oral involvement with a cytotoxic T-cell phenotype, and is strongly associated with EBV. As shown in this series, HVLPD may show aggressive clinical behaviour.

Hidroa vacuniforme: informe de un caso con respuesta terapéutica favorable a cloroquina y tacrolimus ungüento al 0.1% / Hydroa vacciniforme: One case report and response to tacrolimus oinment and antimalarials

La hidroa vacuniforme es una rara fotodermatosis idiopática de curso crónico-recidivante, especialmente durante los meses de verano que comienza en la niñez, para desaparecer hacia la adolescencia. Se caracteriza por la aparición repetida de pápulo-vesículas, cubiertas por costras hemáticas en la piel fotoexpuesta, que van dejando cicatrices varioliformes permanentes. Se describe una presentación clásica estival de naturaleza benigna y otra grave, vinculada con infiltración linfocítica maligna.

Hydroa vacciniforme is a rare idiopathic photodermatosis with a chronic relapsing course, especially during the summer months beginning in childhood, to disappear into adolescence. It is characterized by the repeated occurrence of papulovesicles covered by hematic scabs on the photo exposed skin, that leave permanent varioliform scars. A classic benign presentation and another severe and malignant form with lymphocytic infiltration are described.

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.

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.

Linfoma cutáneo angiocéntrico de células T que semeja hidroa vacciniforme / Angiocentric cutaneous lymphoma in a 14 year old female adolescent

Introducción. Los linfomas cutáneos son procesos linfoproliferativos malignos de células B o T, con una variedad de manifestaciones clínicas, sin que exista enfermedad extracutánea al momento del diagnóstico. Existen reportes de casos de lesiones cutáneas previas, determinadas por fotosensibilidad, que se asocian a linfomas cutáneos. Se presenta el caso de un paciente femenino con diagnóstico de linfoma cutáneo angiocéntrico que semeja hidroa vacciniforme. Caso clínico. Adolescente femenino de 14 años de edad, que ingresó al Hospital General Dr. Aurelio Valdivieso del Estado de Oaxaca en el mes de octubre de 2003. Cuadro actual: 8 meses previos a su ingreso con dermatosis en áreas expuestas al sol, caracterizadas por vesículas y pápulas, que evolucionaron a úlceras y necrosis en miembros torácicos y piernas, con hepatoesplenomegalia y edema centrofacial. La histopatología de la lesión reveló vasculitis angiocéntrica, angiodestructiva, con linfocitos atípicos y paniculitis. Se definió el diagnóstico de linfoma cutáneo angiocéntrico. Se instaló quimioterapia combinada con involución de su dermatosis y compromiso sistémico. Conclusión. El presente caso cumple con criterios clínico e histopatológicos de linfoma cutáneo angiocéntrico, con adecuada respuesta a quimioterapia de combinación, sobre todo basada en antraciclinas.

Introduction. The cutaneous lymphoma is a malignant lymphoproliferative process of B or T, cells with a variety of clinical manifestation without extracutaneous disease at the time of diagnosis. There are case reports in the literature which have associated prior cutaneous injuries, with cutaneous lymphoma as determined by photosensitization. The present case report is of a female patient with diagnosis of cutaneous angiocentric lymphoma which resembled hydroa vacciniform. Case report. Female adolescent 14 year of age who was admitted to Hospital General Dr. Aurelio Valdivieso in the State of Oaxaca, Mexico. Eight months prior to hospital admission she presented dermatosis in areas exposed to the sun, characterized by vesicles, papules which evolved to ulcers and necrosis in arms and legs, she also presented with hepatosplenomegaly and facial edema. The histopatology of the lesions revealed angiodestructive, angiocentric vasculitis with atypical lymphocytes and paniculitis. The diagnosis of angiocentric cutaneous lymphoma was made. The patient was treated with combined chemotherapy with involution of her dermatosis and systemic findings. Conclusion. The present case fulfills the histopathology and clinical criteria of an angiocentric cutaneous lymphoma, with suitable response to combined chemotherapy based on anthracyclines.