CASE REPORT: Brooke-Spiegler Syndrome

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 lesions revealed cylindroma with foci of spiradenoma. discussion: brooke-spiegler syndrome (bss) caused by various mutations of the cylindromatosis gene (cyld), a tumor suppressor gene resulting in multiple cylindromas and trichoepitheliomas. malignant transformation may develop within solitary cylindromas, but more common in multiple variant and requires careful followup surveillance.