Lewandowsky-Lutz epidermodysplasia verruciformis

Verruciform epidermodysplasia of Lewandowsky-Lutz (syn. verrucosis generalisata) is a rare disease, in some cases familial. Autosomal recessive or X-linked inheritance is assumed. It usually occurs in childhood, manifested by widespread, mainly warty rashes, which have some features depending on the localization. Thus, on the face and neck they are similar to flat warts, on the limbs, especially on the distal parts, they look like common warts with a tendency to group, stripe arrangement, fusion with the formation of large warty foci. On the trunk, flat, sharply defined plaques and foci resembling verrucous lichen predominate. The color of the elements varies from the color of normal skin to pinkish-red, reddish-brown. Unlike other types of warts, which disappear spontaneously within a few months or years, epidermolysplasia verruciformis has a permanent course, with a high risk of malignant transformation mainly in lesions located on exposed parts of the body.

Pathomorphology. The picture resembles that of a flat juvenile wart. Acanthosis with thickening of epidermal outgrowths and hyperkeratosis are noted. However, vacuolization of cells in the upper parts of the epidermis is more pronounced, the vacuoles are significantly larger, which is typical for this disease. No special changes are found in the dermis.

Histogenesis. Warts in epidermodysplasia verruciformis are caused by various types of human papillomavirus, but mainly by viruses of types 3, 5 and 8. Several types of the virus are often detected in the same patient, but there are cases of isolation of only one of them. Various disorders of cellular immunity have been identified, mainly a decrease in the number and suppression of the function of T-helpers, but this is not observed in all patients. The most oncogenic viruses are HPV-5, HPV-8 and HPV-14.