title: [Brooke-Spiegler syndrome].
abstract: Brooke Spiegler syndrome is a rare genodermatosis characterized by an association of multiple trichoepitheliomas and cylindromas, sometimes accompanied by other adnexal tumors (spiradenoma, mila), we report two family cases of Brooke Spiegler syndrome. In the first case: a 31 years-old woman, with multiple trichoepitheliomas involving in the naso-genal areas, with multiple cylindromas of the scalp. The second case regarding the brother: 37 years-old, presented with multiple trichoepitheliomas of the face, associated with milia. No neoplastic tumor was identified. A co-existence of adnexal tumors was found in the other members of the family. Brooke Spiegler syndrome is an autosomial dominant disease with variable penetrance, it's characterized by a family history of trichoepitheliomas, with other adnexal tumors. The course of the disease is characterized by a multiplication of lesions, a malignant transformation was reported, but remains rare. Brooke Spiegler syndrome is usually a benign disease, but patients with this syndrome should be explored for malignancy. A family study is indicated.
authors.affiliation: Service de dermatologie,Hôpital Charles, Nicolle Tunis, Tunisie.
authors.forename: Inès
authors.initials: I
authors.lastname: Zaraa
authors.forename: Faten
authors.initials: F
authors.lastname: Zeglaoui
authors.forename: Bacima
authors.initials: B
authors.lastname: Fazaa
authors.forename: Nadia
authors.initials: N
authors.lastname: Ezzine
authors.forename: Nadia
authors.initials: N
authors.lastname: Elfekih
authors.forename: Rachida
authors.initials: R
authors.lastname: Zermani
authors.forename: Sara
authors.initials: S
authors.lastname: Ben Jilani
authors.forename: Mohamed Ridha
authors.initials: MR
authors.lastname: Kamoun
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D008297:Male; D018294:Neoplasms, Adnexal and Skin Appendage / Q000235:genetics*; D009386:Neoplastic Syndromes, Hereditary / Q000235:genetics*; D012878:Skin Neoplasms / Q000235:genetics*; D013577:Syndrome