[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.