Tehran University of Medical Sciences Basic & Clinical Cancer Research 2228-6527 3 1 2011 02 01 65 69 EN Mostafa Mirshams Shahshahani Department of Dermatology,Tehran university of medical sciences Maedeh Rayati Damavandi Department of Dermatology,Tehran university of medical sciences Kambiz Kamyab Department of Pathology, Tehran university of medical sciences Gholam-Reza Maghsoudnia Department of Plastic Surgery, Tehran university of medical sciences Zeinab Aryianian Department of Dermatology,Tehran university of medical sciences 2011 09 06 2011 09 06 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. https://bccr.tums.ac.ir/index.php/bccrj/article/view/10 https://bccr.tums.ac.ir/index.php/bccrj/article/download/10/10