Familial Epidermodysplasia Verruciformis of Lewandowsky and Lutz

Abstract

Epidermodysplasia verruciformis of Lewandowsky and Lutz (EV) occurred as an autosomal recessive, sex-limited trait in six girls of one Chinese family. Except for one patient, all developed EV lesions at the age of 7 or 8 years. This patient with delayed onset and milder cutaneous lesions was given away for adoption at the age of 1 month to avoid contact with the affected sisters. Clinical and histological pictures were classical of EV but in addition, the cases showed marked pigmentary changes simulating arsenical dermatosis, xeroderma pigmentosum, and Bowen's disease. Histologically, there was extensive vacuolation of the epidermal cells, involving even the midmalpighian layer. Intranuclear viral particles previously found in this condition were also demonstrated. The relationship between this genodermatosis, presence of intranuclear viral infection in skin lesions, and increased frequency of malignant changes in this disease is discussed.