A case report of a female patient with Brooke-Spiegler syndrome
Prikaz slučaja bolesnice sa Brooke-Spiegler sindromom
Abstract
Brooke-Spiegler syndrome is a rare autosomal dominant hereditary disease with variable penetration. The syndrome is manifested by the appearance of three types of tumors: multiple cylindromas, trichoepitheliomas and spiradenomas. Tumors can cover the entire scalp in the form of turban, so Turban tumor is usually a synonym for this disease. We presented a patient with a typical clinical picture of Brooke-Spiegler syndrome, who has a positive family history (mother, two sisters, brother and niece). Recommended therapy for this syndrome is surgical treatment. She did not accept surgical treatment, because she was afraid of losing her hair.
Brooke-Spiegler sindrom je retko autozomno dominantno nasledno oboljenje s varijabilnom penetracijom gena. Sindrom se manifestuje pojavom tri tipa tumora: multipli cilindromi, trihoepiteliomi i spiradenomi. Tumori mogu da prekriju čitavu poglavinu u vidu turbana, tako da se kao sinonim za ovu bolest koristi i naziv Turban tumor. U radu je iznet prikaz slučaja bolesnice, starosti 48 godina, s tipičnom kliničkom slikom Brook-Spiegler sindroma, koja ima pozitivnu porodičnu anamnezu. Brook-Spiegler sindrom ispoljio se kod majke, dve sestre, brata i sestričine naše bolesnice. Kao terapija ovog sindroma preporučuje se hirurško lečenje, koje je naša bolesnica odbila iz ličnih razloga zbog straha od gubitka kose.
Keywords:
Brooke-Spiegler syndrome / Cylindroma / Trichoepithelioma / Turban tumors / Brooke-Spiegler sindrom / cilindrom / trihoepiteliom / Turban tumorSource:
Medicinski časopis, 2017, 51, 2, 59-62Publisher:
- Srpsko lekarsko društvo - Okružna podružnica Kragujevac, Kragujevac
Collections
Institution/Community
PharmacyTY - JOUR AU - Savčić, Gordana AU - Paravina, Mirjana AU - Koravović, Mladen PY - 2017 UR - https://farfar.pharmacy.bg.ac.rs/handle/123456789/2947 AB - Brooke-Spiegler syndrome is a rare autosomal dominant hereditary disease with variable penetration. The syndrome is manifested by the appearance of three types of tumors: multiple cylindromas, trichoepitheliomas and spiradenomas. Tumors can cover the entire scalp in the form of turban, so Turban tumor is usually a synonym for this disease. We presented a patient with a typical clinical picture of Brooke-Spiegler syndrome, who has a positive family history (mother, two sisters, brother and niece). Recommended therapy for this syndrome is surgical treatment. She did not accept surgical treatment, because she was afraid of losing her hair. AB - Brooke-Spiegler sindrom je retko autozomno dominantno nasledno oboljenje s varijabilnom penetracijom gena. Sindrom se manifestuje pojavom tri tipa tumora: multipli cilindromi, trihoepiteliomi i spiradenomi. Tumori mogu da prekriju čitavu poglavinu u vidu turbana, tako da se kao sinonim za ovu bolest koristi i naziv Turban tumor. U radu je iznet prikaz slučaja bolesnice, starosti 48 godina, s tipičnom kliničkom slikom Brook-Spiegler sindroma, koja ima pozitivnu porodičnu anamnezu. Brook-Spiegler sindrom ispoljio se kod majke, dve sestre, brata i sestričine naše bolesnice. Kao terapija ovog sindroma preporučuje se hirurško lečenje, koje je naša bolesnica odbila iz ličnih razloga zbog straha od gubitka kose. PB - Srpsko lekarsko društvo - Okružna podružnica Kragujevac, Kragujevac T2 - Medicinski časopis T1 - A case report of a female patient with Brooke-Spiegler syndrome T1 - Prikaz slučaja bolesnice sa Brooke-Spiegler sindromom VL - 51 IS - 2 SP - 59 EP - 62 DO - 10.5937/mckg51-14558 ER -
@article{ author = "Savčić, Gordana and Paravina, Mirjana and Koravović, Mladen", year = "2017", abstract = "Brooke-Spiegler syndrome is a rare autosomal dominant hereditary disease with variable penetration. The syndrome is manifested by the appearance of three types of tumors: multiple cylindromas, trichoepitheliomas and spiradenomas. Tumors can cover the entire scalp in the form of turban, so Turban tumor is usually a synonym for this disease. We presented a patient with a typical clinical picture of Brooke-Spiegler syndrome, who has a positive family history (mother, two sisters, brother and niece). Recommended therapy for this syndrome is surgical treatment. She did not accept surgical treatment, because she was afraid of losing her hair., Brooke-Spiegler sindrom je retko autozomno dominantno nasledno oboljenje s varijabilnom penetracijom gena. Sindrom se manifestuje pojavom tri tipa tumora: multipli cilindromi, trihoepiteliomi i spiradenomi. Tumori mogu da prekriju čitavu poglavinu u vidu turbana, tako da se kao sinonim za ovu bolest koristi i naziv Turban tumor. U radu je iznet prikaz slučaja bolesnice, starosti 48 godina, s tipičnom kliničkom slikom Brook-Spiegler sindroma, koja ima pozitivnu porodičnu anamnezu. Brook-Spiegler sindrom ispoljio se kod majke, dve sestre, brata i sestričine naše bolesnice. Kao terapija ovog sindroma preporučuje se hirurško lečenje, koje je naša bolesnica odbila iz ličnih razloga zbog straha od gubitka kose.", publisher = "Srpsko lekarsko društvo - Okružna podružnica Kragujevac, Kragujevac", journal = "Medicinski časopis", title = "A case report of a female patient with Brooke-Spiegler syndrome, Prikaz slučaja bolesnice sa Brooke-Spiegler sindromom", volume = "51", number = "2", pages = "59-62", doi = "10.5937/mckg51-14558" }
Savčić, G., Paravina, M.,& Koravović, M.. (2017). A case report of a female patient with Brooke-Spiegler syndrome. in Medicinski časopis Srpsko lekarsko društvo - Okružna podružnica Kragujevac, Kragujevac., 51(2), 59-62. https://doi.org/10.5937/mckg51-14558
Savčić G, Paravina M, Koravović M. A case report of a female patient with Brooke-Spiegler syndrome. in Medicinski časopis. 2017;51(2):59-62. doi:10.5937/mckg51-14558 .
Savčić, Gordana, Paravina, Mirjana, Koravović, Mladen, "A case report of a female patient with Brooke-Spiegler syndrome" in Medicinski časopis, 51, no. 2 (2017):59-62, https://doi.org/10.5937/mckg51-14558 . .