Acquired epidermodysplasia verruciformis or generalized verrucosis? A clinical and virological comparative study.

Few articles have described the difference between epidermodysplasia verruciformis (EV) and generalized verrucosis (GV). This study aimed to analyze the clinical findings and virology of the two diseases. The study enrolled patients diagnosed with EV and GV by clinical and histopathological findings. The demographic information, clinical manifestation, treatment, and therapeutic outcome were analyzed. All of the biopsy specimens of enrolled patients were sent for ß-human papillomavirus (HPV) DNA detection. A total of 13 cases were included in the present study, including five EV cases and eight GV cases. Legs and feet were significantly affected in GV cases (p = 0.035). All acquired EV cases had immunocompromised conditions, while only three GV cases could be identified as having an immunocompromised history. All EV cases were detected with ß-HPV infection, especially HPV5, but none of the GV cases were found to be infected with ß-HPV. The therapeutic response was refractory and often relapsed after discontinuation of treatment in both groups.

Epidermodysplasia verruciformis: successful treatment with squaric acid dibutylester.

Epidermodysplasia verruciformis (EV) is a rare disorder characterized by disseminated cutaneous warts in predisposed patients who are highly susceptible to genus ß-papillomavirus infections. We present the case of a 40-year-old lymphocytopenic woman with a balanced chromosomal translocation and a 25-year history of refractory EV that was successfully treated with squaric acid dibutylester (SADBE) contact immunotherapy.

Epidermo displasia verruciforme asociado a carcinoma de piel y anexos. Reporte de caso / Dysplasia verruciformis associated epidermolysis a skin carcinoma and annexes. I reported case

Se describe el caso de un paciente varón de 39 años, natural y procedente de Cajamarca, agricultor, sin antecedentes de importancia, evaluado en el Instituto Nacional de Enfermedades Neoplásicas (INEN) con tiempo de enfermedad de 20 años caracterizado por lesiones eritematoescamosas, hiperqueratosicas, hiper e hipopigmentadas en cuello, tórax, abdomen, miembros superiores e inferiores, pabellon auricular izquierdo, cuero cabelludo y región frontal, clínicamente compatibles con Epidermodisplasia Verruciformis.

We describe the case of a male patient aged 39, from and natural of Cajamarca, farmer, no history of importance, which goes to National Institute of Neoplastic Diseases (INEN) with a disease duration of 20 years characterized by erythematous lesions, hyper- keratosis, hyper-and hypopigmented lesions in neck, chest, abdomen, arms and legs, left ear, scalp and frontal region, clinically compatible with epidermodysplasia verruciformis.

Epidermodisplasia verruciforme: reporte de un caso / Epidermodysplasia verruciformis: report of a case

La epidermodisplasia verruciforme (EV) es una genodermatosis rara y hereditaria que ocasiona una susceptibilidad incrementada a infecciones por serotipos espec¡ficas de papilomavirus humano, se manifiesta con lesiones cut neas similares a verrugas planas, pitiriasis versicolor y queratosis seborreicas. Existen dos fenotipos de EY, benigno y maligno, lo cual va a condicionar el pron¢stico de la enfermedad; hasta el momento sigue siendo una condici¢n de dif¡cil manejo que requiere un seguimiento de las lesiones por su riesgo de malignizacion.

Epidermodysplasia verruciformis is a rare, inherited genoderma-tosis resulting in increased susceptibility to infections by specific serotypes of HPY, manifesting with skin lesions similar to flat warts, pityriasis versicolor and seborrheic keratoses. There are two phenotypes, benign and malignant, which will condition the prognosis of the disease so far remains an unwieldy condition that requires monuoring of its risk lesions of malignant.

Acquired epidermodysplasia verruciformis: a comprehensive review and a proposal for treatment.

BACKGROUND: Epidermodysplasia verruciformis (EV) is a rare genodermatosis characterized by immunologic abnormalities, disseminated human papilloma virus infection, and early development of skin cancers. Acquired forms have been rarely reported and usually occur with immunosuppression. The therapeutic management of the acquired forms is not standardized, and several therapies have been tried, with variable outcomes. OBJECTIVES: To provide updated clinical and experimental information on the treatment of acquired EV. METHODS: A Medline literature search was performed for relevant Medical Subject Heading terms, reviewing publications on strategies for management of acquired EV. We also report a case successfully treated using a combination of photodynamic therapy and oral retinoids. CONCLUSION: Data from the literature show that a standardized approach to this condition is lacking; the combination treatment chosen in our case may be proposed because it led to an excellent clinical outcome and a long-lasting remission.

Epidermodisplasia verruciforme: reporte de una serie de casos / Epidermodysplasia verruciformis: report of a case series

La epidermodisplasia verruciforme es una genodermatosis rara que condiciona una susceptibilidad anormal a infecciones por el virus del papiloma humano, presentando lesiones cutáneas con potencial oncogénico. Objetivo: determinar la frecuencia de epidermodisplasia verruciforme y caracterizas los hallazgos demográficos, clínicos y terapéuticos de los pacientes que acudieron a la Cátedra de Dermatología del Hospital de Clínicas, entre los años 2003, 2012. Material y métodos: se realizó un estudio descriptico de pacientes con diagnóstico clínico e histopatológico de epidermodisplasia verruciforme. Resultados: se encontraron cinco casos. Todos los pacientes iniciaron el cuadro en la infancia y presentaron lesiones tipo pitiriasis versicolor y verrugas planas. Todos refirieron exposición solar crónica y familiares de primer grado con la misma enfermedad. La neoplasia más frecuente fue al enfermedad de Bowen, seguida del carcinoma epidermoide infiltrante. Los tratamientos incluyeron cirugía, crioterapia y 5-fluorouracilo tópico...

Epidermodysplasia verruciformis is a rare genodermatosis characterized by an abnormal susceptibility to human papiloma virus infection, developing cutaneous lesions with oncogenic potential. Objective: To determine the frequency, clinical demographic and therapeutic characteristics of patients who attend to the Dermatology Department of the Hospital de Clinicas between 2003 and 2012. Methods: A descriptive study of patients with clinical and histopathological diagnosis of epidermodysplasia verruciformis was carried out. Results: Five cases were found. Childhood-onset of disease, pityriasis versicolor-like lesions and flat warts were seen in all cases. All patients reported chronic sun exposure and first-degree relatives with the same disease. BowenÆs disease was the most frequent neoplasm, followed by invasive squamous cell carcinoma. Treatment modalities included surgery, cryotherapy and topical 5 fluorouracil. Discussion: Consistently to the infrequent nature of the disease, only five cases were diagnosed in the years. Unlike other studies, BowenÆs disease was the predominant neoplasm...

Autosomal dominant epidermodysplasia verruciformis: a clinicotherapeutic experience in two cases.

Epidermodysplasia verruciformis (EV) is a rare genodermatosis characterized by a unique susceptibility to cutaneous infection by a group of phylogenetically related human papilloma viruses (HPVs). These patients show a defect in cell-mediated immunity specific toward the causative HPVs that lead to lifelong disease. The defect is usually inherited as autosomal recessive trait and presents clinically with plane warts, pityriasis versicolor-like lesions and reddish verrucous plaques. Dysplastic and malignant changes in the form of actinic keratoses, Bowen's disease and squamous cell carcinoma (SCC) are common but metastasis occurs rarely. A totally effective treatment against EV is as yet highly desirable. Two siblings having autosomal dominant EV presented with multiple actinic keratoses in addition to classic lesions. One of them had also developed well-differentiated SCC over forehead with metastases to regional lymph nodes. They were treated with combination of excision of small malignant/premalignant lesions, topical 5-flurouracil and sun protection. Additionally, elective excision/grafting of large SCC was performed after chemotherapy/radiotherapy in patient with metastatic SCC. Oral acitretin (25 mg/day) was of benefit in the other patient. Overall clinicotherapeutic experience in both the patients is discussed here.

Epidermodysplasia verruciformis associated with myelodysplastic syndrome: an intriguing association.

BACKGROUND: Epidermodysplasia verruciformis (EV) is a rare genodermatosis characterized by massive infection with human papillomaviruses (HPVs) and development of skin cancer. Myelodysplastic syndromes (MDSs) are a group of chronic conditions that involve dysplastic hematopoiesis, peripheral blood cytopenias, and a high incidence of progression into leukemia. METHODS: We describe the intriguing association of these two premalignant conditions (EV and MDS) in one patient. These diagnoses were confirmed by histopathologic examination and cytogenetic abnormalities of bone marrow cells. RESULTS: The patient presented initially with clinical features typical of EV and impairment of cell-mediated immunity. In the skin lesions, HPVs 23 and 25 were identified by nested polymerase chain reaction. Six years later, he had recurrent episodes of mucosal bleeding with fever, weakness, and fatigue. At this time, severe refractory anemia and neutropenia were observed, and bone marrow smears showed hypercellularity with abnormal dysplastic megakaryocytes. The cytogenetic pattern showed abnormalities involving trisomy of chromosomes 8 and 21. The patient received a diagnosis of the indolent subtype of MDS. CONCLUSIONS: Through the observation of our patient and review of the literature, we hypothesized that the pathomechanisms, including the role of oncogenes and cytokines, are connected to the progression to malignancy in these settings.

Epidermodysplasia verruciformis occurring in a patient with human immunodeficiency virus: a case report.

Epidermodysplasia verruciformis (EV) is an uncommon dermatosis associated with human papillomavirus (HPV) infection in association with defects in cell-mediated immunity. Malignant transformation to squamous cell carcinoma has been associated with lesions caused by HPV-5, HPV-8, and HPV-14. Clinically, the disease may be confused with verruca plana, seborrheic keratosis, and pityriasis versicolor. We present an unusual case of EV occurring in a human immunodeficiency virus (HIV)-positive man and discuss the clinical and histologic findings. Clinically, the patient had 1- to 3-mm hypopigmented smooth macules covering the entire body. Histopathologic examination of the skin biopsy results demonstrated enlarged keratinocytes with prominent blue-gray cytoplasm and clumping of keratohyalin granules within the granular layer of the epidermis. Although EV typically is viewed as a disease of childhood, sometimes presenting in patients with a family history of the disease, it rarely may be seen in immunocompromised adults.

Treatment of a patient with epidermodysplasia verruciformis carrying a novel EVER2 mutation with imiquimod.

Epidermodysplasia verruciformis (EV) is a rare disorder characterized by widespread human papillomavirus infection and malignant transformation. EV may be caused by mutations of the genes EVER1 or EVER2, which are located on the EV1 locus, 17q25. We describe a patient with EV and a novel homozygous gene mutation of EVER2 gene who was treated successfully with topical imiquimod.

Epidermodysplasia verruciformis treated using topical 5-aminolaevulinic acid photodynamic therapy.

We describe a 65-year-old woman who had had wart-like lesions on the hands, lower arms and forehead for about 45 years. She had already had several basal cell carcinomas excised. Histological study, electron microscopy and in situ hybridization [human papilloma virus (HPV)-types 5/8/12/14/19-23/25/36] of skin biopsies confirmed a diagnosis of epidermodysplasia verruciformis (EV). Photodynamic therapy (PDT) was performed using a 20% 5-aminolaevulinic acid ointment applied for 6 h to the lesions and irradiating using an incoherent light source (lambda = 580-740 nm, 160 mW/cm2, 160 J/cm2). Following PDT, blistering and crusting of the lesions occurred, but these healed completely within 2-3 weeks without scarring, and the cosmetic result was excellent. Six months after PDT a skin biopsy was taken. In situ hybridization was positive for HPV type 8 in skin which was clinically and histologically normal. Twelve months after PDT a few lesions had recurred on the hands. Although permanent cure of EV cannot be achieved by any therapy at present and single lesions continue to appear in this patient, topical PDT might result in better control of HPV-induced lesions.

Multiple metachronous skin squamous cell carcinomas and epidermodysplasia verruciformis in the head region: a human papilloma virus-associated disease.

Data from a young adult man with epidermodysplasia verruciformis (EV) and multiple metachronous spinaliomas in the head and neck region are presented. Diagnosis of this rare, human papilloma virus (HPV)-associated disease was based on: (1) Typical skin lesions, including viral warts, verruca plana-type lesions and pityriasis versicilor; (2) typical histological features, including "foamy giant keratinocytes"; (3) evidence of HPV 5, 8 and 20 in pityriasis versicilor-like lesions; (4) a cellular immunodeficiency due to a relative T-helper-cell deficit. No specific treatment of EV is known, so that therapy concentrates on early removal of spinaliomas and treatment of intercurrent infections. Since EV patients have numerous benign skin lesions and frequently develop metastatic and non-metastatic carcinomas, molecular changes of HPV during carcinogenesis can be studied.