Epidermodisplazya Verrüsiformisli Iki Kardes Olguda Erken Yasta Skuamöz Hücreli Karsinom Gelisimi/Early Development of Squamous Cell Carsinoma in Two Sister Cases with Epidermodysplasia Verruciformis

Özet. Epidermodisplazya verrüsiformis (Lewandowsky-Lutz sendromu), multipl verruka plana, pitriyazis versikolor benzeri lezyonlar, hücresel immünitede bozukluk ve özellikle günefle maruz kalan bölgelerde deri kanseri geliflimi ile karakterize nadir görülen bir hastaliktir. En sik otozomal resesif geçifl gösterir. Epidermodisplazya verrüsiformisli hastalarda en sik geliflen kanser türü skuamöz hücreli kanserlerdir. Deri kanserlerinin % 90'dan fazlasnda human papilloma virüs bulunur. En sik tespit edilen human papilloma virusler 5,8 ve 47 tipleridir. Epidermodisplazya verrüsiformis tedavisi basilica önleyici yöntemlerden oluflur. Fotokoruma tedavide önemlidir. Bu makalede, verruka plana, pitriyazis versikolor benzeri lezyonlar ve günefle maruz kalan bölgelerde skuamöz hücreli karsinom geliflen iki kz kardefl hastal¤n nadir görülmesi ve erken yaflta malinite geliflimi nedeni ile sunulmufltur. (Türkderm 2010; 44: 92-5). Anahtar Kelimeler: Epidermodisplaz ya verrüsiformis, human papilloma virüs, skuamöz hücreli karsinom. Summary. 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. (Turkderm 2010; 44: 92-5). Key Words: Epidermodysplasia verruciformis, human papilloma virus, squamous cell carcinoma.

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