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Early Development of Squamous Cell Carsinoma in Two Sister Cases with pidermodysplasia Verruciformis

Author(s): Ömer Çalka | Serap Güneş Bilgili | Necmettin Akdeniz | İrfan Bayram

Journal: Turkderm
ISSN 1019-214X

Volume: 44;
Issue: 2;
Start page: 92;
Date: 2010;
Original page

Keywords: Epidermodysplasia verruciformis | human papilloma virus | squamous cell carcinoma

Epidermodysplasia verruciformis (Lewandowsky-Lutz syndrome) is an uncommon disease characterized by multiple plane warts, pityriasis versicolor-like lesions, defects of cell-mediated immunity, and tendency to develop skin malignancies, primarily on sun-exposed areas. Most commonly it is inherited as an autosomal recessive trait. Squamous cell carcinoma is the most common type of skin cancer found in patient with epidermodysplasia verruciformis. Human papilloma virus 5, 8, and 47 are found in more than 90% of epidermodysplasia verruciformis skin cancers. Treatment for epidermodysplasia verruciformis consists largely of preventive measures. Photoprotection remains essential for management. In this report, two sister case of epidermodisplasia verruciformis with plane warts, pityriasis versicolor-like lesions, and squamous cell carcinomas on sun-exposed areas of skin was presented for it is a rarely encountered disease and associated with early development of malignancy.
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Tango Jona
Tangokurs Rapperswil-Jona