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Tuberous Sclerosis Complex, Two Case Reports

Year 2007, Volume: 14 Issue: 2, 123 - 127, 01.04.2007

Birgül Tepe Hamdi Özcan Muammer Seyhan

Abstract

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

Keywords

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References

  • Rosser T, Panigrahy A, McClintock W. The diverse clinical manifestations of tuberous sclerosis complex: a review. Semin Pediatr Neurol. 2006;13: 27-36.
  • Levine D, Barnes P, Korf B, Edelman R. Tuberous sclerosis in the fetus: second- trimester diagnosis of subependymal tubers with ultrafast MR imaging. AJR.2000;175: 1067-9.
  • Devlin LA, Shepherd CH, Crawford H, Morrison PJ. Tuberous sclerosis complex: clinical features, diagnosis,and prevalence within Northern İreland. Dev Med Child Neurol. 2006; 48: 495-9.
  • Harper JI. Tuberous sclerosis complex. In: Champion RH, Burton JL, Burns DA, Breathnanh SM eds. Textbook of Dermatology. 6th ed. United Kingdom: Blackwell Science 1998:384-8.
  • http://www.emedicine.com/derm/topic438.htm Nambi R. Tuberous Sclerosis. eMedicine. 2006.
  • Morris BS, Garg A, Jadhav PJ. Tuberous sclerosis : A presentation of less-commonly encountered stigmata. Austral Radiol. 2002; 46: 426-30.
  • Suwattee P, Walsh C, Ho N. Proteus syndrome and tuberous sclerosis: any genetic association? J Am Acad Dermatol 2004; 50: P89.
  • Odom RB, James WD, Berger TG. Tuberous sclerosis (Epiloia, Bourdneville’s disease). In: Andrew’s Dıseases of the Skın. 9th ed. Philadelphia: WB Saunders Company. 2000:687-9.
  • Lin AN, Carter DM. Tuberous sclerosis. In: Moschella SL, Hurley HJ eds. Dermatology. 3th ed. Philadelphia: W.B.Saunders Company 1992:1339-43.
  • Patel SB, Shah SS, Goswami K, Shah N, Pandhi S. Case report: Tuberous sclerosis- it’s varied presentation. Ind J Radiol Imag. 2004; 14: 423-5.
  • Marcotte L, CrinoPB. The neurobiology of the tuberous sclerosis complex. Neuromolecular Med. 2006; 8:531-46.

Tuberoz Skleroz Kompleksi, İki Olgu Sunumu

Year 2007, Volume: 14 Issue: 2, 123 - 127, 01.04.2007

Birgül Tepe Hamdi Özcan Muammer Seyhan

Abstract

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

Keywords

Tuberoz skleroz Adenoma sebaseum Koenon tümörü

References

  • Rosser T, Panigrahy A, McClintock W. The diverse clinical manifestations of tuberous sclerosis complex: a review. Semin Pediatr Neurol. 2006;13: 27-36.
  • Levine D, Barnes P, Korf B, Edelman R. Tuberous sclerosis in the fetus: second- trimester diagnosis of subependymal tubers with ultrafast MR imaging. AJR.2000;175: 1067-9.
  • Devlin LA, Shepherd CH, Crawford H, Morrison PJ. Tuberous sclerosis complex: clinical features, diagnosis,and prevalence within Northern İreland. Dev Med Child Neurol. 2006; 48: 495-9.
  • Harper JI. Tuberous sclerosis complex. In: Champion RH, Burton JL, Burns DA, Breathnanh SM eds. Textbook of Dermatology. 6th ed. United Kingdom: Blackwell Science 1998:384-8.
  • http://www.emedicine.com/derm/topic438.htm Nambi R. Tuberous Sclerosis. eMedicine. 2006.
  • Morris BS, Garg A, Jadhav PJ. Tuberous sclerosis : A presentation of less-commonly encountered stigmata. Austral Radiol. 2002; 46: 426-30.
  • Suwattee P, Walsh C, Ho N. Proteus syndrome and tuberous sclerosis: any genetic association? J Am Acad Dermatol 2004; 50: P89.
  • Odom RB, James WD, Berger TG. Tuberous sclerosis (Epiloia, Bourdneville’s disease). In: Andrew’s Dıseases of the Skın. 9th ed. Philadelphia: WB Saunders Company. 2000:687-9.
  • Lin AN, Carter DM. Tuberous sclerosis. In: Moschella SL, Hurley HJ eds. Dermatology. 3th ed. Philadelphia: W.B.Saunders Company 1992:1339-43.
  • Patel SB, Shah SS, Goswami K, Shah N, Pandhi S. Case report: Tuberous sclerosis- it’s varied presentation. Ind J Radiol Imag. 2004; 14: 423-5.
  • Marcotte L, CrinoPB. The neurobiology of the tuberous sclerosis complex. Neuromolecular Med. 2006; 8:531-46.
There are 11 citations in total.

Details

Primary Language Turkish
Journal Section Articles
Authors

Birgül Tepe This is me

Hamdi Özcan This is me

Muammer Seyhan This is me

Publication Date April 1, 2007
Published in Issue Year 2007 Volume: 14 Issue: 2

Cite

APA Tepe, B., Özcan, H., & Seyhan, M. (2007). Tuberoz Skleroz Kompleksi, İki Olgu Sunumu. Journal of Turgut Ozal Medical Center, 14(2), 123-127.
AMA Tepe B, Özcan H, Seyhan M. Tuberoz Skleroz Kompleksi, İki Olgu Sunumu. J Turgut Ozal Med Cent. April 2007;14(2):123-127.
Chicago Tepe, Birgül, Hamdi Özcan, and Muammer Seyhan. “Tuberoz Skleroz Kompleksi, İki Olgu Sunumu”. Journal of Turgut Ozal Medical Center 14, no. 2 (April 2007): 123-27.
EndNote Tepe B, Özcan H, Seyhan M (April 1, 2007) Tuberoz Skleroz Kompleksi, İki Olgu Sunumu. Journal of Turgut Ozal Medical Center 14 2 123–127.
IEEE B. Tepe, H. Özcan, and M. Seyhan, “Tuberoz Skleroz Kompleksi, İki Olgu Sunumu”, J Turgut Ozal Med Cent, vol. 14, no. 2, pp. 123–127, 2007.
ISNAD Tepe, Birgül et al. “Tuberoz Skleroz Kompleksi, İki Olgu Sunumu”. Journal of Turgut Ozal Medical Center 14/2 (April 2007), 123-127.
JAMA Tepe B, Özcan H, Seyhan M. Tuberoz Skleroz Kompleksi, İki Olgu Sunumu. J Turgut Ozal Med Cent. 2007;14:123–127.
MLA Tepe, Birgül et al. “Tuberoz Skleroz Kompleksi, İki Olgu Sunumu”. Journal of Turgut Ozal Medical Center, vol. 14, no. 2, 2007, pp. 123-7.
Vancouver Tepe B, Özcan H, Seyhan M. Tuberoz Skleroz Kompleksi, İki Olgu Sunumu. J Turgut Ozal Med Cent. 2007;14(2):123-7.