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SHR Neuro Cancer Cardio Lipid Metab Microb

Parente, JN; Schettini, AP; Massone, C; Parente, RT; Schettini, RA.
Do you know this syndrome?.
An Bras Dermatol. 2009; 84(5):547-549 Doi: 10.1590/S0365-05962009000500019 (- Case Report) [OPEN ACCESS]
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Co-authors Med Uni Graz
Massone Cesare
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Abstract:
Brooke-Spiegler syndrome is an autosomal dominant inherited disease with predisposition to cutaneous adnexal neoplasms, most commonly cylindromas and trichoepitheliomas. Its onset is in the second or third decades of life. The histopathological exams of the lesions revealed a plethora of benign adnexal neoplasms, showing apocrine, follicular, and sebaceous differentiation. The treatment can be performed by excisional surgery, laser, cryotherapy, electrofulguration and dermabrasion. Due to the risk of malignancy, there is the need for clinical follow-up and genetic counseling.
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