A 62-year-old female presented with numerous soft tissue lesions of her scalp and bilateral preauricular region. Several of these have been biopsied or removed with a diagnosis of cylindromas. Cylindromas are benign tumors with a differentiation towards apocrine sweat glands that increase in number and size throughout life. Multiple scalp cylindromas may coalesce and cover the entire scalp, res...


 Introduction
 Cylindroma is an appendageal tumour of the skin that usually benign in nature, arising from pluripotent stem cells follicular-sebaceous-apocrine unit. Usually, multiple cylindromas are a component Brooke-Spiegler syndrome (BSS) or occur only as lesions familial cylindromatosis (FC). The site occurrence tumours this condition scalp.
 Case Report
 Here, we repo...

Brooke-Spiegler Syndrome (BSS) is a rare genodermatosis characterized by the progressive formation of adnexal skin tumors in the scalp and face, mainly trichoepitheliomas, cylindromas, and spiradenomas. It has also been associated with salivary glands neoplasms. It is due to mutations in the tumor suppressor gene cylindromatosis (CYLD gene) localized on chromosome 16q12-q13. Around 93 mutations...

espanolEl sindrome de Brooke-Spiegler (SBS) es una genodermatosis poco frecuente, herencia autosomica dominante, con penetrancia y expresividad variables, que predispone a la produccion neoplasias anexiales. En clinica se caracteriza por aparicion simultanea progresiva cilindromas, tricoepiteliomas y, en ocasiones, espiradenomas. El SBS produce mutaciones el gen CYLD1 localizado cromosoma 16 fo...

The mathematical equation for Separation of the binary aqueous solution is developed by using the SpieglerKedem theory. The characteristics of a B-9 hollow fibre module of Du Pont are determined by using these equations and their results are compared with the experimental results of Ohya et al. The agreement between these results is found to be excellent. Keywords—Binary aqueous solution, model...

Brooke-Spiegler syndrome (BSS; OMIM 605041) is a rare monogenic skin disease characterized by the development of appendage tumors caused mutations in cylindromatosis (CYLD) gene. Despite described phenotypes and reports underlying CYLD mutations, it has been difficult to establish genotype–phenotype correlations BSS. We recently investigated two BSS pedigrees (Hungarian with Bukovinian origin A...