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...

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...

Cylindroma is a benign skin appendageal tumor arising from pluripotent stem cells in the follicle. Multiple cylindroma typically occurs as a component of Brooke-Spiegler syndrome or as the only skin lesion of familial cylindromatosis. We herein report an unusual case of multiple cylindroma involving the trunk without any other features of Brooke-Spiegler syndrome and no family history supportin...

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 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 reviewe...

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...

In the past decade the molecular basis of many inherited syndromes has been unravelled. This article reviews the clinical and genetic aspects of inherited syndromes that are characterised by skin appendage neoplasms, including Cowden syndrome, Birt-Hogg-Dube syndrome, naevoid basal cell carcinoma syndrome, generalised basaloid follicular hamartoma syndrome, Bazex syndrome, Brooke-Spiegler syndr...

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.