title: Pedigree of multiple benign adnexal tumours of Brooke-Spiegler type.
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.
authors.affiliation: Department of Medicine (Dermatology), Royal Hobart Hospital, Tasmania, Australia.
authors.forename: Helen
authors.initials: H
authors.lastname: Saunders
authors.forename: Paul
authors.initials: P
authors.lastname: Tucker
authors.forename: Tricia
authors.initials: T
authors.lastname: Saurine
authors.forename: Frances
authors.initials: F
authors.lastname: Watkins
mesh_terms: D000293:Adolescent; D000328:Adult; D001315:Australia; D001707:Biopsy, Needle; D005260:Female; D005500:Follow-Up Studies; D020022:Genetic Predisposition to Disease; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D018294:Neoplasms, Adnexal and Skin Appendage / Q000235:genetics* / Q000473:pathology*; D010375:Pedigree; D018570:Risk Assessment; D012878:Skin Neoplasms / Q000235:genetics* / Q000473:pathology*