Epidermodisplazya Verr�siformisli �ki Karde� Olguda Erken Ya�ta Skuam�z H�creli Karsinom Geli�imi

�alka, �mer; G�ne� Bilgili, Serap; Akdeniz, Necmettin; Bayram, �rfan

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Epidermodisplazya Verr�siformisli �ki Karde� Olguda Erken Ya�ta Skuam�z H�creli Karsinom Geli�imi [Turkderm-Turk Arch Dermatol Venereol]

Turkderm-Turk Arch Dermatol Venereol. 2010; 44(2): 92-95

Epidermodisplazya Verr�siformisli �ki Karde� Olguda Erken Ya�ta Skuam�z H�creli Karsinom Geli�imi

�mer �alka¹, Serap G�ne� Bilgili², Necmettin Akdeniz¹, �rfan Bayram³
¹Y�z�nc� Y�l �niversitesi T�p Fak�ltesi, Deri ve Z�hrevi Hastal�klar Anabilim Dal�, Van, T�rkiye
²�pekyolu Devlet Hastanesi, Deri ve Z�hrevi Hastal�klar Klini�i, Van, T�rkiye
³Y�z�nc� Y�l �niversitesi T�p Fak�ltesi, Patoloji Anabilim Dal�, Van, T�rkiye

Epidermodisplazya verr�siformis (Lewandowsky-Lutz sendromu), multipl verruka plana, pitriyazis versikolor benzeri lezyonlar, h�cresel imm�nitede bozukluk ve �zellikle g�ne�e maruz kalan b�lgelerde deri kanseri geli�imi ile karakterize nadir g�r�len bir hastal�kt�r. En s�k otozomal resesif ge�i� g�sterir. Epidermodisplazya verr�siformisli hastalarda en s�k geli�en kanser t�r� skuam�z h�creli kanserlerdir. Deri kanserlerinin % 90�dan fazlas�nda human papilloma vir�s bulunur. En s�k tespit edilen human papilloma virusler 5,8 ve 47 tipleridir. Epidermodisplazya verr�siformis tedavisi ba�l�ca �nleyici y�ntemlerden olu�ur. Fotokoruma tedavide �nemlidir. Bu makalede, verruka plana, pitriyazis versikolor benzeri lezyonlar ve g�ne�e maruz kalan b�lgelerde skuam�z h�creli karsinom geli�en iki k�z karde� hastal��n nadir g�r�lmesi ve erken ya�ta malinite geli�imi nedeni ile sunulmu�tur.

Anahtar Kelimeler: Epidermodisplaz ya verr�siformis, human papilloma vir�s, skuam�z h�creli karsinom

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

�mer �alka¹, Serap G�ne� Bilgili², Necmettin Akdeniz¹, �rfan Bayram³
¹Deparment Of Dermatology, Y�z�nc� Y�l University, Van, Turkey
²Deparment Of Dermatology, Ipekyolu State Hospital, Van, Turkey
³Deparment Of Patology, Y�z�nc� Y�l University, Van, Turkey

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.

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

Sorumlu Yazar: �mer �alka, T�rkiye
Makale Dili: T�rk�e

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