Brooke-spiegler syndrome
نویسنده
چکیده
BrookeSpiegler syndrome is an uncommon autosomal dominant disorder, only few cases have been reported. Herein, we report a patient with this rare condition. A 26 year old woman with Brooke-Spiegler syndrome (BSS) has multiple cutaneous adenxal neoplasms on her face and scalp. Clinical features, histopathogical spectrum, pathogenesis, malignant transformation and treatment modalities are reviewed. Correspondence: Dr. Osama Nour Eldin MD, Department of Dermatology, Farwaniya Hospital, Kuwait. Nabeel Najem et,al.
منابع مشابه
Brooke-Spiegler Syndrome: a case report
Brooke-Spiegler syndrome is a rare autosomal recessive disease characterized by adnexal neoplasms, particularly trichoepithelioma, cylindroma, and occasionally spiradenoma, which usually develop in second to third decades of life. We report this syndrome in a 16-year-old woman with tumors on face and scalp.
متن کاملBrooke-Spiegler Syndrome: A Case Report
Page 19 Abstract Brooke-Spiegler syndrome (BSS) is a rare, inherited, autosomal-dominant genodermatosis characterized by the development of multiple adnexal cutaneous tumors including spiradenomas, cylindromas, spiradenocylindromas, trichoepitheliomas, epidermoid cysts, and milia. We present a case of Brooke-Spiegler syndrome with possible malignant transformation of a benign tumor. Brooke-Spie...
متن کاملBrooke-Spiegler Syndrome: A Rare Entity
Brooke-Spiegler syndrome is a rare entity. It is an autosomal dominant syndrome in which multiple trichoepitheliomas, cylindromas, or other adnexal tumors are seen. Very few cases of Brooke-Spiegler syndrome are reported in the literature. We came across a 40 -year-old female in which multiple trichoepitheliomas and cylindromas were seen on scalp. In view of clinical history and histopathologic...
متن کاملMalignant cylindroma in a patient with Brooke-Spiegler syndrome
Malignant cylindroma (cylindromatous carcinoma, cylindrocarcinoma) is the malignant counterpart of benign cylindroma. It is a rare neoplasm, more often developing in the setting of multiple preexisting benign neoplasms. Herein we present an additional case of malignant transformation of the cylindroma diagnosed in an 83-year-old patient with known Brooke-Spiegler syndrome.
متن کاملA novel germline mutation in the CYLD gene in a Slovak patient with Brooke-Spiegler syndrome.
The authors report a 64-year-old female with Brooke-Spiegler syndrome who presented with multiple cutaneous nodules and tumors mostly involving the scalp. Histopathological examination of one of the lesions located in a periauricular area revealed a typical cylindroma. In some neoplastic nodules ductal differentiation and occasional bilayered glands composed of the dark abluminal basal/myoepith...
متن کامل