title: Brooke-Spiegler syndrome.
abstract: 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 describes a 26-year-old woman who presented to the dermatology department of Qaem Hospital with tumoral lesions on her scalp, face, and forearm. Her father and younger brother were also affected. On examination, several round-to-oval skin-colored papules with a smooth pearly surface measuring 2 to 6 mm in diameter were seen on the mid-face, particularly in the nasolabial folds, the upper lip. Tumors and nodules seen on the scalp were pinkish red, dome-shaped, and to some extent, pedunculated with surface telangiectasia and induration. Histopathology of the facial papules showed trichoepithelioma while that of a scalp nodule showed cylindroma.
authors.affiliation: Qaem Hospital, Mashad University of Medical Sciences, Mashad, Iran. pouran_layegh@yahoo.com
authors.forename: Pouran
authors.initials: P
authors.lastname: Layegh
authors.forename: Nourieh
authors.initials: N
authors.lastname: Sharifi-Sistani
authors.forename: Mohammad
authors.initials: M
authors.lastname: Abadian
authors.forename: Toktam
authors.initials: T
authors.lastname: Moghiman
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D054020:Lasers, Gas / Q000627:therapeutic use; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery*; D013577:Syndrome