Anais Brasileiros de Dermatologia
On-line version ISSN 1806-4841
PARENTE, José Napoleão Tavares et al. Do you know this syndrome?. An. Bras. Dermatol. [online]. 2009, vol.84, n.5, pp. 547-549. ISSN 1806-4841. http://dx.doi.org/10.1590/S0365-05962009000500019.
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.
Keywords : Adenoid cystic carcinoma; Sebaceous gland neoplasms; Skin neoplasms.