Abstract

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 histopathological examination it was diagnosed as Brooke-Spiegler syndrome. We report this case because of its rarity.

Highlights

  • Brooke-Spiegler syndrome was reported for the first time in 1842 by Ancell [1]

  • More than 50 cases of Brooke-Spiegler syndrome are reported in the literature so far

  • Features favouring cylindroma included islands of basaloid cells surrounded by hyalinized material

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Summary

Introduction

Brooke-Spiegler syndrome was reported for the first time in 1842 by Ancell [1]. It is an autosomal dominantly inherited syndrome, characterised by multiple skin appendageal tumors [2]. More than 50 cases of Brooke-Spiegler syndrome are reported in the literature so far

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