Tuberoskleroz Kompleksi
Tuberoskleroz (TS) başlıca mental retardasyon, epilepsi ve adenoma sebaseum triadı ile karakterize otozomal dominant geçişli bir hastalıktır. Tüm triad hastaların ancak üçte birinde saptanabilmektedir. Tuberoskleroz kompleksi (TSK), başlıca santral sinir sistemi, deri, retina, böbrek ve kalbi tutan bir hamartomatozistir. Tuberosklerozlu hastalarda böbrek tutulumu ile ilişkili patolojiler; angiomyolipom (%70–80), renal kist (%20) ve çok nadiren kanserler olup en sık görülen nörolojik bulgu ise epileptik nöbetlerdir. Bu yazıda, yüzde sivilce yakınması ile dermatoloji polikliniğine başvuran deri bulguları ile tuberoskleroz kompleksi tanısı düşünülen, radyolojik incelemelerle ek olarak kortikal tuber ve anjiyomiyolipom saptanan bir olgu sunuma değer bulunmuştur.
Tuberous sclerosis complex
Tuberous sclerosis (TS) is an autosomal dominant disorder characterized mainly by mental retardation, epilepsy and adenoma sebasemia triad. All triads can be detected in only one third of the patients. Tuberous sclerosis complex (TSC) is a hamartomatosis that mainly carries the central nervous system, skin, retina, kidney and heart. Pathologies associated with renal involvement in patients with tuberous sclerosis; angiomyolipomas (70-80%), renal cysts (20%) and very rarely cancers. The most common neurological finding is epileptic seizures In this article, we present a case report of a patient who applied to dermatological clinic with acne compleints and diagnosed as a tuberous sclerosis complex with skin findings, cortical tuber and angiomyolipoma in addition to radiological examinations.
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