Tuberous Sclerosis Complex, Two Case Reports

Yıl 2007, Cilt: 14 Sayı: 2, 123 - 127, 01.04.2007

Birgül Tepe Hamdi Özcan Muammer Seyhan

Öz

Tuberous sclerosis known as Epiloia and Bourneville's disease is a neurocutane syndrome, which is resulted from the programmed hyperplasia of ectodermal and mesodermal cells. The term tuberous sclerosis complex is now widely used. The term of the complex remarks variable multisystemic involvement and genetic heterogeneity of the disease. The disease showing different variations from the age of onset and the severity have classical triads ofepilepsy, mental retardation and adenoma sebaceum. The management of the disease is made according to the affected organ. This rarely seen disease was discussed because of the two different cases admitted to our clinics and detailed information about the cutaneous findings are given. Key Word: Tuberous sclerosis, Adenoma sebaceum, Koenen tumor

Anahtar Kelimeler

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Tuberoz Skleroz Kompleksi, İki Olgu Sunumu

Öz

Epiloia veya Bourneville hastalığı olarak da bilinen tuberoz skleroz, ektodermal ve mezodermal hücrelerin programlanmış hiperplazisinden kaynaklanan nörokutan bir sendromdur. Günümüzde tuberoz skleroz kompleksi terimi daha yaygın olarak kullanılmaktadır. Kompleks terimi, hastalığın genetik heterojenitesi yanında değişik multisistemik tutulumlarına da işaret etmektedir. Başlangıç yaşı ve şiddeti çok geniş varyasyonlar gösteren hastalığın klasik triadı; epilepsi, mental retardasyon ve adenoma sebaseum'dur. Tedavi etkilenen organa yöneliktir. Oldukça nadir görülen hastalık, kliniğimize başvuran iki farklı olgu nedeni ile tartışılmış ve uyarıcı olması gereken deri bulgularına ayrıntılı olarak yer verilmiştir. Anahtar kelimeler: Tuberoz skleroz, Adenoma sebaseum, Koenon tümörü

Anahtar Kelimeler

Tuberoz skleroz, Adenoma sebaseum, Koenon tümörü

Kaynakça

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