Pityriasis Versicolor-like Lesions Research Articles

Epidermodysplasia Verruciformis: A Case Report and a Brief Review

Epidermodysplasia verruciformis is an uncommon disorder that is transmitted in an autosomal recessive manner. It is characterized by increased susceptibility to human papillomavirus infection, which presents with hypo- or hyperpigmented macular lesions, pityriasis versicolor-like lesions, and an early tendency to transform into skin cancer. We present a case of a 40-year-old male with complaints of verrucous lesions of the hands and feet. Histopathology was suggestive of EV.

Epidermodysplasia Verruciformis: A Case Report

Epidermodysplasia verruciformis (EDV) is a rare, inherited disorder that is characterized by an increased susceptibility to infection with specific human papilloma viruses (HPV) and a greater propensity for developing malignant skin tumors.
 A 26 years old Nepalese male patient presented with complaints of multiple asymptomatic raised warty lesions on bilateral hands and multiple light colored flat to raised lesions present over the neck, trunk, bilateral extremities sparing the face and genitals.A skin biopsy was performed which revealed hyperkeratosis, focal parakeratosis, acanthosis, keratinocytes were swollen and irregularly shaped, had abundant basophilic cytoplasm and contained numerous round basophilic keratohyaline granules. Nuclei were large, round and empty with marginal distribution of chromatin, some were pyknotic.
 Epidermodysplasiaverruciformis is a rare inherited disorder spread by HPV. It is characterized by hypopigmented or hyperpigmented macular lesions, pityriasis versicolor-like lesions and an early tendency to develop skin malignancies. The management of EDV includes early diagnosis, sun protection, life-long observation for the malignant transformation and therapeutic modalities like acitretin, imiquimod, topical retinoids, cryotherapy, etc.

Epidermodysplasia verruciformis as a manifestation of ARTEMIS deficiency in a young adult

Abstract This study is the first to report epidermodysplasia verruciformis (EV) associated with ARTEMIS deficiency in a young adult. This broadens the clinical scope of ARTEMIS deficiency, and provides a new genetic etiology for susceptibility to EV.

Epidermodysplasia Verruciformis Associated with Astrocytoma, Mantle Lymphoma and Hepatitis B Virus Infection

Epidermodysplasia verruciformis (EV) is a lifelong, rare autosomal recessive genetic hereditary skin disorder characterized by a unique susceptibility to human papilloma virus, associated with a high risk of malignant transformation. 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. No definitive treatment against Epidermodysplasia verruciformis has been found yet. Extracutaneous cancers reported in Epidermodysplasia verruciformis are intestinal adenocarcinoma, plasmablastic lymphoma and leiomyosarcoma. Mantle cell lymphoma is uncommon under the age of 40 years and rare in patients under the age of 30 years. Here we are reporting a 19 year male patient with Epidermodysplasia verruciformis, astrocytoma and pulmonary tuberculosis who presented with symptoms of weight loss, indigestion and burning epigastric pain four months duration, Upper GIT endoscopy showed a gastric mass with active peptic ulcers. Biopsy revealed Mantle cell lymphoma. We investigated further and found to have immunodeficiency and hepatitis B virus infection. EV patients should receive regular follow-up for possible cutaneous or extracutaneous malignancy.

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.

EVER2 Deficiency is Associated with Mild T-cell Abnormalities

Epidermodysplasia verruciformis (EV) is a rare genodermatosis characterized by persistent flat warts or pityriasis versicolor-like lesions caused by betapapillomaviruses (EV-HPVs). Autosomal recessive EVER1 and EVER2 deficiencies account for EV in most patients. The mechanisms by which mutations in these partners of the Zinc transporter ZnT1 impair host defense against EV-HPVs are still poorly understood. Keratinocytes of EVER-deficient patients display an alteration of zinc homeostasis and an enhanced proliferative activity. Since EVER proteins are highly expressed in T lymphocytes, we aimed to assess the impact of EVER2 deficiency on T-cell development and function. We studied circulating lymphocyte populations in three adult EV patients sharing the same EVER2 mutation (T150fsX3). We found a normal count of CD4(+) and CD8(+) T cells and a normal proliferative capacity in response to anti-CD3 stimulation. However, we observed a significant increase of memory CD4(+) and effector memory CD8(+) T cells, a bias of the TCR Vαβ and Vγδ repertoires and an increase of skin-homing CD4(+) T-cell subsets. Our findings suggest that EVER2-deficient patients display mild T-cell abnormalities. It remains unclear whether these abnormalities result from EVER deficiency, chronic EV-HPV infection, or both.

Epidermodysplasia verruciformis and human immunodeficiency virus infection

Epidermodysplasia verruciformis and human immunodeficiency virus infection

Epidermodisplasia verruciforme: apresentação clínica com variadas formas de lesões

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.

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.

Ehlers‐Danlos syndrome – vascular type (ecchymotic variant): cutaneous and dermatopathologic features

Ehlers-Danlos syndrome - vascular type, the only lethal form, is rarely reported in dermatology literature. It is characterized by translucent, atrophic skin, easy bruising, arterial, intestinal and/or uterine fragility manifesting as varicose veins, aneurysms and vascular/visceral/uterine rupture. As its dermatopathologic features are not well elucidated, diagnosis is often made after a catastrophic complication or at autopsy. This 36 year-old non-consanguineous male had brown-black plaques with atrophy and frequent ulceration over legs and dorsal feet and tortuous varicose veins around ankles for the past 15 years. Perivenous skin was translucent and hypopigmented. He had multiple and ecchymotic keloids and small atrophic, pityriasis versicolor-like lesions over trunk. He did not have hypermobile/hyperextensible skin and joints and showed no systemic or investigative abnormality. Histopathologic features of atrophic lesion included blood extravasation in atrophic epidermis/dermis, focal clustering and dilatation of blood vessels, malformed vessel walls, abundant hemosiderin in the dermis and homogenously stained/whorled patterned collagen especially around blood vessels. Pathology of keloidal lesion showed new collagen and vascular fragility. These histopathologic features appear of diagnostic value especially in patients who have compatible clinical findings but cannot afford confirmation by biochemical testing for abnormal synthesis of type III procollagen or identification of mutations in the COL3A1 gene.

Epidermodysplasia verruciformis: a model for understanding the oncogenicity of human papillomaviruses.

The first evidence for the oncogenic potential of human papillomaviruses (HPVs) was obtained through the study of epidermodysplasia verruciformis (EV). This rare skin disease is characterized by disseminated, refractor, pityriasis versicolor-like lesions as well as flat wart-like lesions, and by the development of skin carcinomas in about 30% of the patients. EV is a multifactorial disease involving genetic, immunological and extrinsic (actinic) factors, in addition to infection with specific HPV types. A number of HPVs (at least 15 types) have been characterized in benign EV lesions. HPV DNA sequences are regularly detected in EV carcinomas but, in contrast to benign lesions, the types associated with cancers are usually restricted to HPV-5 and, less frequently, HPV-8, an HPV-5-related type. HPV-5 genomes are usually found as free monomeric or oligomeric DNA molecules in EV carcinomas, and frequently contain deletions. This is in contrast with HPV DNA sequences in genital cancers, which are often integrated into the host DNA. Evidence for the transcription of HPV-5 genomes in primary and metastatic EV carcinomas has recently been obtained. The available data indicate that HPV-5 and some HPV-5-related types have an oncogenic potential and play a role in the malignant transformation of EV lesions. Infection by these HPVs must be considered a major risk factor for the development of cancers in EV patients. EV HPV DNA sequences have only rarely been detected in premalignant or malignant lesions of the skin in the general population. This further stresses the role of genetic, immunological and extrinsic factors in the abnormal susceptibility of EV patients to a set of specific HPV types.

Aspectos clínicos da epidermodisplasia verruciforme

FUNDAMENTOS: A epidermodisplasia verruciforme (EV) é genodermatose rara, caracterizada por infecção disseminada por tipos específicos de vírus papiloma humano (HPV), desenvolvimento de tumores cutâneos malignos e distúrbios imunológicos. OBJETIVOS: Correlacionar aspectos clínicos em 13 doentes com EV, na tentativa de contribuir para melhor conhecimento da enfermidade. MÉTODOS:Avaliação clínica de 13 doentes com EV durante o período de três anos. O diagnóstico clínico foi confirmado pelo exame histopatológico e imuno-histoquímico. RESULTADOS: A EV teve início na infância com lesões de verruga plana-símile e/ou máculas eritematosa na face e região cervical. A consangüinidade foi observada na maioria dos doentes (12/13). Clinicamente, o polimorfismo das lesões foi intenso, caracterizado por lesões de verruga plana-símile, pitiríase versicolor-símile, máculas eritematosas e lesões de queratose seborréica-símile. A transformação maligna das lesões foi observada em oito doentes (62%). O crescimento tumoral provocou perda tecidual importante em 50% dos casos, e em 25% foi registrado óbito pelas metástases. CONCLUSÃO: A EV apresenta alta incidência familiar e provável transmissão autossômica recessiva. O intenso polimorfismo clínico das lesões não afeta o couro cabeludo e mucosas. A apresentação clínica "maligna" foi a mais freqüente (62%), seguida pela "benigna" (23%) e "mista" (15%). Os tumores cutâneos malignos são freqüentes, múltiplos, destrutivos, geram metástases e provocam morte.

Epidermodysplasia verruciformis-associated papillomavirus infection complicating human immunodeficiency virus disease

Three males infected with the human immunodeficiency virus (HIV) were noted to have extensive flat warts of the face and/or body. In two there were also pityriasis versicolor-like lesions. Biopsies showed foamy, basophilic, distended cytoplasm in granular layer keratinocytes, characteristic of the human papillomavirus types seen in epidermodysplasia verruciformis. DNA hybridization techniques demonstrated the presence of HPV-type 8 in one patient and HPV 5 and 8 in another. Patients with immune suppression due to HIV infection may demonstrate the clinical features of epidermodysplasia verruciformis with the same potentially oncogenic HPV types.

Epidermodysplasia Verruciformis in a Patient with Hodgkin's Disease: Characterization of a New Papillomavirus Type and Interferon Treatment

A new human papillomavirus (HPV) was discovered in disseminated, macular, pityriasis versicolor-like lesions on the skin of the neck, face, scalp, and pubic region of a 42-year-old male suffering from Hodgkin's disease. Histopathology revealed features characteristic of epidermodysplasia verruciformis (ev). In contrast to classical ev, the lesions were almost exclusively seen in previously irradiated and UV-exposed skin areas. Papillomavirus capsid antigen was demonstrated with the genus-specific antiserum and the patient's serum, which had IgM and IgG antibody titers. HPV DNA was isolated from biopsies and cloned into the vector pIC20H. It proved to be related to ev-associated viruses, showing 23% cross-hybridization with DNA of the closest relative HPV14. The new HPV type was named HPV46. The genome was physically mapped and colinearly aligned with HPV8 DNA to establish its gene organization. Interferon treatment of the patient did not significantly change the clinical picture nor was the concentration of viral DNA per lesion affected. However, no virus capsid antigen was detectable after starting treatment.

Twenty-One Years of Follow-Up Studies of Familial Epidermodysplasia verruciformis

21 years of follow-up study of a family with epidermodysplasia verruciformis (e.v.) have shown that members of one family can be infected with different human papillomaviruses (HPVs), either HPV 3 or HPV 4, and sometimes with both. The clinical picture resembled disseminated flat warts in cases induced by HPV 3, whereas in those caused by HPV 4 there were flat red or red-brownish plaques and depigmented pityriasis versicolor-like lesions. Malignancies developed only in family members infected with HPV 4, whereas the cases due to HPV 3 ran a more benign and slowly progressive or stationary course. There were also abortive and regressive cases, and the 3 children in whom the wart-like lesions did not recur after removal had an unimpaired cell-mediated immunity (CMI). In all cases of e.v., irrespective of the inducing virus, CMI was low, which seems to be an important factor in the pathogenesis of the disease. Humoral antibodies directed specifically against HPV 3 were present in the majority of the cases, mainly those infected with HPV 3.