Epidermodysplasia verruciformis associated with idiopathic CD4+ lymphopenia / Epidermodisplasia verruciforme asociada a linfopenia idiopática de CD4+

Abstract Epidermodysplasia verruciformis (EV) is an autosomal recessive disease of the skin commonly associated with EVER1 and EVER 2 mutations, and is characterized by high susceptibility to infections associated with certain types of human papillomavirus called EV-PVH. Patients have warty lesions on the skin of varying characteristics and are often associated with skin cancer, with a strong association being found with EVER1 and EVER2 mutation gene. The case presented below concerns an absolute CD4+ lymphopenia, and establishes the hypothesis of a possible mutation of the RHOH gene as its origin.

Resumen La epidermodisplasia verruciforme (EV) es una enfermedad de la piel autosómica recesiva, relacionada con la mutación EVER1 y EVER2, caracterizada por alta susceptibilidad a infecciones asociadas a ciertos tipos de papillomavirus humano llamadas EV-PVH. Los pacientes presentan lesiones verrucosas en la piel de características variadas y muchas veces asociadas a cáncer de piel no melanocítico, encontrándose una fuerte asociación con la mutación del gen EVER1 y EVER2. El caso que se presenta a continuación documenta linfopenia absoluta de CD4+ por lo que se plantea la hipótesis de una posible mutación del gen RHOH como etiología.

Epidermodisplasia verruciforme / Epidermodysplasia verruciformis

La epidermodisplasia verruciforme (EV) es una genodermatosis rara, caracterizada por el desarrollo de verrugas planas y lesiones similares a pitiriasis versicolor, asociada a infección crónica por Virus Papiloma Humano. Se reporta el caso de EV. Mujer de 49 años con múltiples lesiones verrucosas de larga evolución distribuidas en las cuatro extremidades y antecedente familiar de cuadro similar. Biopsia a los 19 años evidenció verrugas sin atipia. Nuevo estudio histológico informó carcinoma escamoso, decidiéndose extirpación de lesiones. La clínica inicia en edades tempranas, con elevado riesgo de transformación maligna. No existe tratamiento específico. Es esencial la fotoprotección estricta y manejo precoz de lesiones para prevención de progresión a carcinoma.

Epidermodysplasia verruciformis (EV) is a rare genodermatosis, characterized by the development of plane warts and pityriasis versicolor-like lesions, associated with chronic infection caused by the Human Papilloma virus. An EV case is reported. 49 year-old woman with multiple long standing warty lesions distributed on the four limbs and family background of similar cases. Biopsy at 19 years old evidenced warts without atypia. New histological study reported squamous cell carcinoma, deciding the removal of the lesions. The clinic begins at early age, with high risk of malignant transformation. There is no specific treatment. Strict photoprotection and early management of the lesions is essential to prevent the progression to carcinoma.

Acquired epidermodysplasia verruciformis in a renal transplant recipient - Case report

A 24-year-old male patient, who underwent kidney transplant six years ago due to Lupus nephritis, for the last two years presented asymptomatic erythematous scaly plaques on the abdomen and areas exposed to light. Post-transplantation immunosuppressive medications included prednisone, mycophenolate sodium and sirolimus. The histopathologic features were typical for epidermodysplasia verruciformis. Epidermodysplasia verruciformis is a rare autosomal recessive genodermatosis with increased susceptibility to specific strains of cutaneous human papilloma virus. The term ''acquired epidermodysplasia verruciformis'' was recently introduced to the literature and describes epidermodysplasia verruciformis occurring in patients with impaired cell-mediated immunity. We report an additional case associated to immunosuppression after kidney transplantation.

Epidermodysplasia Verruciformis-like Skin Eruption in an HIV-positive Patient

Epidermodysplasia Verruciformis-like skin eruption is a rare syndrome that usually occurs in patients with immunodeficiency, predisposing them to infections with human papilloma β virus (HPV). We report here an HIV patientpresenting with this syndrome.

Epidermodysplasia Verruciformis-like skin eruption in HIV-positive patient

Epidermodysplasia Verruciformis-like skin eruption is a rare syndrome that usually occurs in patients with immunodeficiency, predisposing to infections with human papilloma β virus (HPV). We report here a patient with HIV presenting with this syndrome.

Epidermodysplasia verruciformis in a young man with HIV since birth - case report / Epidermodisplasia verruciforme em um jovem com HIV desde o nascimento - relato de caso

Epidermodysplasia Verruciformis is a genodermatosis characterized by susceptibility to infection by specific HPV types (HPV 3/10 and beta-HPVs). It is considered to be the first model in human carcinogenesis induced by HPV. In this report we present a rare case of Epidermodysplasia Verruciformis associated with vertical transmission of HIV. Although most patients with HIV present infections by HPV virus, the frequency of Epidermodysplasia Verruciformis is no greater in patients with HIV because in EV there is a deficiency of specific cellular immunity to infection by some types of HPV, called HPV-related Epidermodysplasia Verruciformis.

A Epidermodisplasia Verruciforme é uma genodermatose que se caracteriza pela susceptibilidade à infecção por tipos específicos de HPV (HPVs 3 e 10 e os beta-HPVs) e é considerada o primeiro modelo no homem de carcinogênese induzida pelo HPV. Neste artigo apresenta-se um caso raro de Epidermodisplasia Verruciforme em um paciente com HIV desde o nascimento. Apesar dos doentes com HIV apresentarem mais infecções pelos vírus HPVs, existem poucos relatos na literatura de Epidermodisplasia Verruciforme no doente com HIV. Isso se explica, pois a Epidermodisplasia Verruciforme é consequência de uma deficiência celular específica à infecção por alguns tipos distintos de HPVs, ditos HPVs relacionados a Epidermodisplasia Verruciforme.

Experiência discente com o cuidado a paciente portador de Epidermodisplasia Verruciforme / Student experience in providing care to a patient with Epidermodysplasia Verruciformis / Experiencia de estudiantes con el cuidado a paciente con Epidermodisplasia Verruciforme

Relato de experiência de situação vivenciada, em 2009, por acadêmicas de enfermagem, durante estágio da disciplina Enfermagem em Doenças Transmissíveis, na enfermaria de dermatologia de um hospital de ensino do interior paulista, em que cuidaram de um paciente com Epidermodisplasia Verruciforme. Esta é uma genodermatose rara, com susceptibilidade à infecção por diferentes tipos de papiloma vírus humanos, ocasionando alterações físicas exacerbadas e impactando o psiquismo da pessoa acometida. A assistência foi baseada na complexidade assistencial indicada pela aplicação do Sistema de Classificação de Pacientes proposto por Perroca. A experiência proporcionou aprendizado no cuidado a paciente portador de doença rara, complexa e de difícil resolução, possibilitando a necessidade de assisti-lo integralmente, percebendo-o além das lesões. Conclui-se que este tipo de experiência muito contribuiu para o crescimento das acadêmicas como futuras profissionais de enfermagem.

Report of an experience of nursing students in providing care to a patient with epidermodysplasia verruciformis, during the internship in the discipline Nursing in Transmittable Diseases, at the dermatology ward of a teaching hospital, located in the state of São Paulo, Brazil, in 2009. This is a rare genodermatosis, susceptible to infection with different types of human papilloma viruses, that cause exacerbated physical changes that impact the psyche of the affected individual. Care delivery was based on the assistance complexity indicated by the application of the Patient Classification System proposed by Perroca. The experience allowed students to learn how to cope with a patient affected by a rare and complex disease, of difficult resolution, and to provide integral care to this individual, perceiving him beyond the lesions. One conclude that the experience has greatly contributed to the growth of the students as future professionals.

Relato de experiencia vivida por estudiantes de enfermería, en 2009, durante la pasantía de la disciplina de Enfermería en Enfermedades Transmisibles, en la sala de dermatología de un hospital universitario del estado de São Paulo, Brasil, donde cuidarán a uno paciente con Epidermodisplasia Verruciforme. Esta es una genodermatosis rara, con susceptibilidad para la infección por diferentes tipos de virus del papiloma humano, causando cambios físicos exasperados e impacto en la psiquis de la persona afectada. La asistencia esté basada en la complejidad de la atención, segundo la aplicación del Sistema de Clasificación de Pacientes propuesto por Perroca. A experiencia proporciono aprendizaje en el cuidado del paciente con una enfermedad rara, compleja y de difícil cura, dando la posibilidad a las estudiantes de asistirlo integralmente, más allá de las lesiones. Concluye-se que este tipo de experiencia contribuyó para el crecimiento de las estudiantes como futuros profesionales.

Epidermodysplasia verruciformis: An unusual malignant transformation.

Epidermodysplasia verruciformis (EV) is a rare, life-long heritable disease caused due to a unique susceptibility to human papilloma virus. The disseminated verrucous lesions and pityriasis versicolor-like lesions persist from early childhood and can transform into a cutaneous malignancy in a fourth of patients. Malignant transformation into syringoid eccrine carcinoma (SEC) has been reported only once so far. SEC is an extremely invasive, rare, locally destructive, slowly growing adnexal tumor. We hereby report the association of EV with SEC in a 29-year-old male.

Epidermodisplasia verruciforme: apresentação clínica com variadas formas de lesões / Epidermodysplasia verruciformis: clinical presentation with varied forms of lesions

Epidermodisplasia verruciforme é uma genodermatose rara caracterizada por infecção disseminada por HPV, de caráter recessivo, com casos ligados ao cromossoma X. É caracterizada clinicamente por lesões maculares hipo ou hiperpigmentadas, lesões pitiríase versicolor like, verrugas planas e desenvolvimento precoce de carcinomas cutâneos. Descreve-se um caso de paciente com quadro clínico exuberante, apresentando todas as formas de lesões desta doença, inclusive presença de carcinoma espinocelular agressivo na face.

Epidermodysplasia verruciformis is a rare inherited skin disorder spread by HPV, with cases linked to chromosome X. It is characterized by hypo- or hyper-pigmented macular lesions, pityriasis versicolor-like lesions and an early tendency to develop skin malignancies. We present a case of epidermodysplasia verruciformis with a variety of lesions such as multiple plane warts, pityriasis versicolor-like lesions and aggressive squamous cell carcinoma on the face.

Caso para diagnóstico / Case for diagnosis

Paciente do sexo feminino, parda, de 27 anos de idade, apresentando há três anos, placas hipocrômicas planas, ovaladas, de superfície áspera, no dorso e membros superiores com aspecto que lembrava pitiríase versicolor. Refere história familiar de um irmão cujas lesões são semelhantes. Exames laboratoriais, incluindo anti-hiv, sem alterações e com histopatológico evidenciando queratinócitos, aumentados de volume, com citoplasma basofílico e microvacuolado ocupando a porção superior da camada espinhosa e a granulosa. Após correlação anátomo-clínica, confirmou-se o diagnóstico de epidermodisplasia verruciforme pitiríase versicolor like.

A 27-year-old mixed-raced (pardo) female patient presented with flat ovate hypochromic plaques with a rough surface on the back and upper limbs, with an aspect resembling pityriasis versicolor. She reports family history involving a brother with similar lesions. Lab tests, including anti-HIV, showed no alterations and a histopathological examination showed enlarged keratinocytes with basophilic and microvacuolated cytoplasm occupying the upper portion of the spinous layer and the granulous layer. After anatomic-clinical correlation, the diagnosis of epidermodysplasia verruciformis pityriasis versicolor like was confirmed.

Expressions of IL-10, IL-23 and CD86 in lesions of epidermodysplasia verruciformis / 中华皮肤科杂志

Objective To measure the expressions of IL-10, IL-23 and CD86 in lesions of epidermodysplasia verruciformis (EV), and to explore the relationship between cellular immune abnormality and EV pathogenesis. Methods Immunohistochemistry was used to measure the expressions of IL-10, IL-23 and CD86 in tissue samples from 10 patients with EV and 10 normal human controls. Results Three cytokines were observed in all the samples of EV, with the expression score ranging from 3 to 6 and expression intensity from moderate to high. However, of the control specimens, only 1 was positive for IL-10 with the expression score being 3, and expression intensity being moderate. Conclusion The pathogenesis of epidermodysplasia verruciformis may be correlated with the expression abnormality of some cytokines secreted by keratinocytes.

A Case of Epidermodysplasia Verruciformis Associated with Squamous Cell Carcinoma and Actinic Keratosis / 대한피부과학회지

Epidermodysplasia verruciformis is a very rare, chronic disease that's characterized by persistent human papillomavirus infection. This type of lesion may transform into malignant skin tumors, usually after the age of 30 years. We report here on a 33-year-old woman who presented with squamous cell carcinoma and actinic keratosis that developed from epidermodysplasia verruciformis. Her malignant skin lesions were effectively treated by surgical excision, cryotherapy and topical application of imiquimod.

A Case of Epidermodysplasia Verruciformis with Cervical and Vulvar Intraepithelial Neoplasia / 대한피부과학회지

Epidermodysplasia verruciformis (EV) is a rare genodermatosis characterized by disseminated infection by human papillomavirus (HPV) and malignant transformation of the lesions in about half of the patients. Immunocompromised states, such as AIDS, renal transplantation, and systemic lupus erythematous, have been associated with EV. A 51-year-old woman taking oral prednisolone for lupus nephritis for about 20 years presented with multiple flat scaly red-brown macules on the whole body for 2 years. On past history, she had an operation on cervical intraepithelial neoplasia 6 months ago and was additionally diagnosed with vulvar intraepithelial neoplasia 3 months ago. Histopathologic examination on the skin lesion revealed typical findings of EV. We, herein, report an unusual case of EV combined with vulvar and cervical intraepithelial neoplasia in a chronic immunocompromised state.

A Case of Epidermodysplasia Verruciformis Related with Agent Orange / 대한피부과학회지

Epidermodysplasia verruciformis(EV) is a rare, often familial disease, associated with human papilloma virus. It is characterized by long-lasting, wide-spread wart-like or pityriasis versicolor-like lesions from early childhood and in about one-third patients, cutaneous carcinomas develop in adult life. We report a case of 57-year-old male with EV, with lesions only on the left forearm and the hand, who had been exposed to Agent Orange during Vietnam War.

Epidermodysplasia Verruciformis in a patient with Systemic Lupus Erythematosus / 대한피부과학회지

Epidermodysplasia verruciformis(EV) is a rare, sometimes familial disorder characterized by chronic infection with EV-specific HPV types. It manifests itself as numerous flat warts, red and brownish macules, and pityriasis versicolor-like lesions. Immunodeficient staies such as AIDS renal transplantation, Hodgkins dis ase, and systemic lupus erythematosus, have been associated with EV. We report a case of a 42-year old female with EV, with lesions on the chest, shoulders, and back, For about the past 2, years, she had been treated with prednisolone for systemic lupus erythematosus. Histopathological findings revealed loose basket-weave-like hyperkeratosis and there were clear large cells arranged in nests in the granular and spinous layers. Their nuclei were shrunken and pyknotic, and the clear cytoplasm was dotted with dispersed keratohyaline granules of different sizes anr shapes. Electron microscopic findings showed aggregates of many viral particles in the nuclei of displastic epidermal keratinocytes. Pan papilloma virus monoclonal Antibody positive nuclei were shown by immunoperoxidase staining. She has been treated with CO, laser and Tretinoin(0.1% ) oin ment. The patient is being monitored for any signs of recurrence for a period of 5months.

A Case of Epidermodysplasia Verruciformis Associated with Bowen's Disease / 대한피부과학회지

We present a case of epidermodysplasia verruciformis a.ssociated with Bowen's disease. Patient was 33 year-old male and his skin lesions showed diffuse flat papules on face and neck, many brownish papules on trunk and papillomatous tumors also appeared un scalp and post auricular area, Skin biopsy findings from the flat papular lesion showed histopathologic features of verruca plana and from the papillomatous lesion showed the feature of Bowen's disease. Electron microscopic finding from the flat papular lesion showed marked aggregates of viral particles around the nuclei of epidermal keratinocytes.