The Brooke-Spiegler syndrome (BSS) is an uncommon autosomal dominant disorder characterized by a high affinity to form multiple adnexal neoplasia (skin appendage tumors), especially trichoepitheliomas and cylindromas, and occasionally spiradenomas, which usually appear in the second or third decade of life. To date, only a few cases with this syndrome have been reported. This case report descri...

introduction : brooke-spiegler syndrome (bss) is a rare autosomal dominant disease characterized by the development of multiple adnexal cutaneous tumors such as cylindromas, trichoepitheliomas and spiradenomas. case description: a 49 years old female patient presented with a history of gradual appearance of multiple nodules situated predominantly on the scalp and face. pathologic examination of...

BrookeSpiegler syndrome (Brooke-Spiegler syndrome; OMIM #605041) is a rare, autosomal dominant inherited monogenic disease caused by mutations in gene CYLD with its different penetrance.
 It clinically manifested the development of multiple neoplasms skin appendages such as spiradenoma, cylindroma, spiradenocylindroma and trichoepithelioma. Several phenotypic variants have been described s...

Trichoepithelioma is an uncommon, benign hamartomatous tumor of the pilosebaceous follicle. Presenting as multiple papules and nodules on face and neck, they pose a significant cosmetic problem in affected individuals. Familial involvement of this dermatosis occurs in an autosomal dominant pattern, the locus being located on chromosome 9p21, which causes multiple facial lesions in family member...