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      Pedigree of multiple benign adnexal tumours of Brooke-Spiegler type.

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          Abstract

          A pedigree of autosomal dominant expression of multiple benign adnexal tumours is presented. Seven cases spanning three generations are discussed. The clinical manifestations of these tumours are quite variable, including multiple papules concentrated on the face, scalp nodules and a large turban tumour. One member of the family had a linear papular eruption involving one half of his body. Histopathology of all lesions demonstrated benign adnexal characteristics, including well-characterized eccrine spiradenomas, trichoepitheliomas and an eccrine cylindroma. The cutaneous tumours occurring in these patients have continued to develop during their lifetimes. The authors propose that this pedigree has phenotypic characteristics consistent with Brooke-Spiegler syndrome.

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          Author and article information

          Journal
          Australas. J. Dermatol.
          The Australasian journal of dermatology
          Wiley
          0004-8380
          0004-8380
          May 2003
          : 44
          : 2
          Affiliations
          [1 ] Department of Medicine (Dermatology), Royal Hobart Hospital, Tasmania, Australia.
          Article
          663
          10.1046/j.1440-0960.2003.00663.x
          12752191

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