A sporadic case of epidermodysplasia verruciformis in a young boy

Epidermodysplasia Verruciformis: A Rare Case Report

“…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].…”

“…With disease progression, some lesions fade and new lesions may emerge on other parts of the body [6]. Findings are confined to the skin and uncommonly appear on the mucous membrane [8].…”

“…Oral retinoids (0.5 mg/kg) used in two sisters with an autosomal recessive pattern showed that cutaneous lesions decreased in size after a short time but a relapse of the lesions occurred after one year [9]. A surgical approach, including electrosurgical removal and cryotherapy, is also used to manage benign and premalignant lesions [6]. Malignant lesions were also surgically treated [6].…”