Epidermodysplasia verruciformis: therapeutic approach with imiquimod

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Epidermodysplasia verruciformis (EV), also known as Lewandowsky-Lutz syndrome, is a rare heritable disease characterized by an unusual susceptibility to infection with specific types of human papillomavirus and a propensity for developing skin malignant tumours [1]. Partial defects in cell-mediated immunity have been demonstrated in this disease. Etiopathogenesis of the disease includes genetic factors, immunologic factors and persistent HPV infections [2,3]. Here, we describe a 13-year-old patient with EV who was treated successfully with imiquimod 5%.

On physical examination, hypopigmented irregular flat wart-like papules on the dorsal hands, involving the fingers (Fig. 3) and on the elbows, and pityriasis versicolor-like macules on the trunk (anterior and posterior), neck and face (sun-exposed skin) (Fig. 1-2) were seen. The lesions are extending into the scalp, which gives the appearance of seborrheic dermatitis.

Biopsy from the lesions showed verruca plana-like lesions with mild hyperkeratosis, hypergranulosis and acanthosis of the epidermis. Keratinocytes of the upper epidermal layers are enlarged with perinuclear vacuolization and a typical blue-grey pallor. The features were consistent with the diagnosis of EV. 

Therapy with electrodesiccation and cryotherapy were unsatisfactory. We started the treatment with imiquimod 5%, one application per day, 3 weeks, followed by one week break. Three treatments were performed with the improvement of the lesions. At two month follow-up, it was decided to resume treatment with imiquimod for even better results.

The use of sunscreen is highly recommended because of the propensity for malignant transformation.