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(a) Multiple hypopigmented macules and papules coalescing to form patches over the forehead. (b) Multiple hypopigmented papules over the right retroauricular area b a
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Epidermodysplasia verruciformis (EV) is a rare disorder that is usually transmitted in an autosomal recessive manner, caused by human papillomavirus which presents with tinea versicolor-like or flat wart-like lesions. It has propensity for malignant transformation, especially squamous cell carcinoma. Here, we present a case of 7 year boy with compl...
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Epidermodysplasia verruciformis (EV), an autosomal recessive genodermatosis, is characterized by persistent cutaneous infections of human papilloma viruses (HPV) of the β-genus. The early clinical manifestations include thin, tinea versicolor- like lesions and flat warts which develop to protruding tumors. Cutaneous malignancies, particularly squam...
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... 1 A higher lifetime cumulative sunlight exposure, X-ray irradiation, and immunologic defects in patients with EV are likely to induce mutations of the tumour suppressor gene protein (p53), leading to the development of skin malignancies in adult patients. 8,9 Transformation into non-melanoma skin cancer occurs in 35-50% of patients between the ages of 40 and 50. 4 Malignant transformation is caused by infection with specific strains of HPV, particularly HPV5 and HPV8. More than 30 EV-associated HPVs have been identified, including HPV5, HPV8, HPV12, HPV14, HPV15, HPV17, HPV19, HPV25, HPV36, HPV38, HPV47, and HPV50. ...
... In order to prevent skin cancer, patients must be educated about the disease's relapsing and persistent course, photoprotection, early detection, and excision of premalignant and malignant lesions with regular follow-up. 3,8 CONCLUSION Acquired EV is a rare entity and can be acquired in patients who are immunodeficient, especially in those who have had a transplant. Recipients of a renal transplant with acquired EV are at high risk of developing non-melanoma skin cancers. ...
The article explains a case presentation of a Epidermodysplasia Verruciformis in a patient with renal transplant which is a rare case.
... 1,2 EVER1 and EVER2 genes belong to the TMC family of proteins (TMC6 and TMC8 account for 75% of the cases) involved in zinc homeostasis and downstream signaling cascades important to the immune system. 1 Most EV cases have been reported to have impaired cellmediated immunity. 1 Given the clinical data available, the patient in this case is presumed to have a sporadic form of EV. 5 B through E. RHOH, MST-1, CORO1A, and IL-7 e Incorrect. Mutations in RHOH, MST-1, CO-RO1A, and IL-7 have been reported in case reports to be associated with EV; however, these mutations are not the most common. ...
... Pharmacological treatments include oral and topical retinoids, interferon, immunotherapy, imiquimod and cimetidine. A surgical approach, including electrosurgical ablation and cryotherapy, is also used to manage benign and premalignant lesions [8]. Malignant lesions have also been treated surgically [8]. ...
... A surgical approach, including electrosurgical ablation and cryotherapy, is also used to manage benign and premalignant lesions [8]. Malignant lesions have also been treated surgically [8]. ...
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.
... In EV, there are mutations in the EVER1 or EVER2 genes located on chromosome 17q25, which, as a result of a defect of cell-mediated immunity, lead to unusual increased sensitivity of the patients to a distinct group of HPV genotypes recognized as EV HPV [1]. Intracellular zinc homeostasis is controlled by a complex of EVER proteins, and zinc transporter proteins have a role in suppressing EV expression [6]. The characteristic feature of EV is a specific defect of cell-mediated immunity that is demonstrated by the inhibition of natural cytotoxicity and the proliferation of T lymphocytes against HPV-infected squamous cells in EV skin lesions [7]. ...
... The characteristic feature of EV is a specific defect of cell-mediated immunity that is demonstrated by the inhibition of natural cytotoxicity and the proliferation of T lymphocytes against HPV-infected squamous cells in EV skin lesions [7]. Ultraviolet (UV) B and X-rays are also important carcinogenic cofactors [6]. Frequent sun exposure, in addition to immunologic defects in patients with EV, is likely to induce mutations of the tumor suppressor gene protein (p53), which leads to the development of skin malignancy in adult patients [8]. ...
... Primary skin lesions are multiform, usually warty, lichenoid, and flat-topped papules [8]. At first, the lesions are confined, hypochromic scaly patches on the face and neck, resembling tinea versicolor [6]. Over a period of time, they increase in number and tend to develop into papules that look like flat warts, pink to brownish in color, a few millimeters in size, with a smooth surface, and later spread to the dorsum of the hands, forearms, knees, legs, and feet [6]. ...
Epidermodysplasia verruciformis (EV) is an uncommon disorder that is transmitted in an autosomal recessive manner. It is characterized by increased susceptibility to human papillomavirus (HPV) 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 36-year-old female with complaints of asymptomatic, multiform lesions over the face, neck, chest, and upper arms. Histopathology was suggestive of EV, and our patient was given oral isotretinoin 20 mg/day and advised strict photoprotection.
