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Case Report

Observations on Four Cases of Brooke–Spiegler Syndrome

1
“Dr. Leventer Centre”, Monetariei Street, no 8, 011216 Bucharest, Romania
2
“Ovidius University”, Mamaia Boulevard, no 124, 900527 Constanta, Romania
*
Author to whom correspondence should be addressed.
Received: 23 August 2020 / Revised: 27 September 2020 / Accepted: 29 September 2020 / Published: 30 September 2020
Background: Brooke–Spiegler Syndrome is a rare genetic autosomal dominant disorder with variable penetrance. Its main feature consists of the development of multiple adnexal tumors that originate from the follicular-sebaceous-apocrine unit, most commonly: cylindromas, trichoepitheliomas and spiradenomas. Case presentation: We present four cases of Brooke–Spiegler Syndrome found in our clinic, as well as their clinicopathological traits and the surgical techniques used in their management. The familial history of three of the presented cases supports the genetic component of the disease. Cylindromas, spiradenomas and trichoepitheliomas coexisted in one of the cases presented. The therapeutic options used were electrocautery, CO2 laser, as well as tumor debulking followed by closure with metal staples. Discussion: The treatment remains a challenge and must be individualized based on the type, location and number of the lesions. Conservative methods such as CO2 laser and tumor debulking accompanied by closure with metal staples remain a viable option taking into account the large number of lesions. As patients usually develop multiple neoplasms throughout their lifetime, repeated procedures may be needed. Conclusion: Considering the few numbers of Brooke–Spiegler syndrome cases in the current literature, the authors report these patients in order to increase awareness and to help establish the most appropriate approach in managing the disease. View Full-Text
Keywords: genodermatosis; adnexal lesions; individualized treatment genodermatosis; adnexal lesions; individualized treatment
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MDPI and ACS Style

Leventer, M.; Coltoiu, C.; Zota, A.; Tebeica, T.; Lisievici, C.; Martinescu, A. Observations on Four Cases of Brooke–Spiegler Syndrome. Reports 2020, 3, 28. https://0-doi-org.brum.beds.ac.uk/10.3390/reports3040028

AMA Style

Leventer M, Coltoiu C, Zota A, Tebeica T, Lisievici C, Martinescu A. Observations on Four Cases of Brooke–Spiegler Syndrome. Reports. 2020; 3(4):28. https://0-doi-org.brum.beds.ac.uk/10.3390/reports3040028

Chicago/Turabian Style

Leventer, Mihaela, Casandra Coltoiu, Alexandra Zota, Tiberiu Tebeica, Carmen Lisievici, and Alina Martinescu. 2020. "Observations on Four Cases of Brooke–Spiegler Syndrome" Reports 3, no. 4: 28. https://0-doi-org.brum.beds.ac.uk/10.3390/reports3040028

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