Introduction

Epidermodysplasia verruciformis (EV), an uncommon lifetime autosomal recessive genodermatosis disorder that affects the immune system, is characterized by increased sensitivity to human papillomavirus (HPV) infection. 

It is found in two forms, sporadic or familial, without a specific race or gender predilection. It features pityriasis Versicolor-like macules, flat wart-like lesions, macules, and seborrheic keratosis-like plaques that begin in childhood mainly on the sun-exposed areas of the face, neck, trunk, and extremities, with an increased risk of the malignant transformation on the sun-exposed areas.

Case report

A 36-year-old, female, daughter of a non-consanguineous marriage couple, presented with complaints of asymptomatic lesions over her face, neck, chest, and upper arm since the age of 16 years. She reported the lesions to start on the chest, which gradually increased in number, spreading to the neck, face, and upper arm. She did not have any history of previous treatment, no family history for the same. She was observed otherwise healthy, with no history of any surgeries. 

Physical examination revealed many multiple brownish, slightly scaly annular macules symmetrically distributed over the neck, chest, and upper arms, multiple erythematous, slightly scaly papules on the neck, and seborrheic keratosis-like lesions on the face. No abnormalities were detected with the hair, nails, mucous membrane, and other systemic examinations. 

A punch skin biopsy, taken from the brownish slightly scaly annular macules over the left outer upper arm, showed epidermal thickening with swollen cells in the upper epidermis. There were nests of large cells with a prominent perinuclear halo and greyish-blue cytoplasm. The stratum corneum showed a basket weave-like pattern, and some epidermal cells showed dysplastic changes. 

The clinical presentation proved to be characteristic, and the histopathological features proved to be diagnostic of epidermodysplasia verruciformis. 

She was started oral retinoid after the Baseline fasting lipid profile and liver function tests results were found normal. Along with this, she was advised of strict photoprotection and started on oral isotretinoin 0.5 mg/kg. 

Discussion

The aim to manage EV is to prevent the progression of benign lesions into malignancy through preventive measures such as genetic counseling, photo protection, and the monitoring of symptoms for the early detection of premalignant and malignant lesions. The patients should use sun block from childhood. 

Pharmacologic treatments of EV include oral and topical retinoids 0.5-1 mg/day being the drug of choice, presently. 

Additionally, surgical approaches, including electrosurgical removal and cryotherapy, can also be used to manage benign and premalignant lesions. Malignant lesions can also be surgically treated. 

Although different treatment modalities are offered against EV, the most important remains the education of the patient, early diagnosis, and excision of the premalignant and malignant lesions. These patients also require regular, life-long follow-up due to the high risk of developing premalignant and malignant lesions.

Source: Alshammari R, Al-Issa A, Ghobara YA. Epidermodysplasia Verruciformis: A Rare Case Report. Cureus. 2020;12(7):e9046. Published 2020 Jul 7. doi:10.7759/cureus.9046