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 CASE REPORT
Year : 2008  |  Volume : 74  |  Issue : 6  |  Page : 632--634

Brooke-Spiegler syndrome


Qaem Hospital, Mashad University of Medical Sciences, Mashad, Iran

Correspondence Address:
Pouran Layegh
Department of Dermatology, Qaem Hospital, Mashad University of Medical Sciences, Mashad
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0378-6323.45109

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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.






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Online since 15th March '04
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