Rare Skin Diseases: Turban Tumor (Brooke-Spiegler Syndrome)

The Brooke-Spiegler syndrome is a rare autosomal dominant disorder characterized by development of multiple skin tumours including multiple cylindromas and trichoepitheliomas. Here we describe a case of 68-year-old man admitted at a consultative appointment into the surgical department of Siberian S...

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Main Authors: V. S. Dmitruk, V. G. Martynova, S. A. Khardikova, K. A. Skrylova, V. I. Shirshkova
Format: Article
Language:Russian
Published: Kemerovo State Medical University 2024-06-01
Series:Фундаментальная и клиническая медицина
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Online Access:https://fcm.kemsmu.ru/jour/article/view/869
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author V. S. Dmitruk
V. G. Martynova
S. A. Khardikova
K. A. Skrylova
V. I. Shirshkova
author_facet V. S. Dmitruk
V. G. Martynova
S. A. Khardikova
K. A. Skrylova
V. I. Shirshkova
author_sort V. S. Dmitruk
collection DOAJ
description The Brooke-Spiegler syndrome is a rare autosomal dominant disorder characterized by development of multiple skin tumours including multiple cylindromas and trichoepitheliomas. Here we describe a case of 68-year-old man admitted at a consultative appointment into the surgical department of Siberian State Medical University with numerous asymptomatic, skin-coloured, dense smooth papulonodular lesions from 2 to 5 mm diameter over the face and with multiple pinkish dense smooth dome-shaped nodules from 1 to 5 cm over the scalp. The patient has noted their appearance since childhood. Earlier, similar lesions have been observed in his father and grandmother. Histopathological examination classified facial lesions as trichoepitheliomas and defined scalp lesions as cylindromas, thereby confirming the diagnosis of Brooke-Spiegler syndrome.
format Article
id doaj-art-54b4539ec0be46a0a60c1a3ae4954e68
institution Kabale University
issn 2500-0764
2542-0941
language Russian
publishDate 2024-06-01
publisher Kemerovo State Medical University
record_format Article
series Фундаментальная и клиническая медицина
spelling doaj-art-54b4539ec0be46a0a60c1a3ae4954e682025-08-20T03:42:38ZrusKemerovo State Medical UniversityФундаментальная и клиническая медицина2500-07642542-09412024-06-019211211710.23946/2500-0764-2024-9-2-112-117430Rare Skin Diseases: Turban Tumor (Brooke-Spiegler Syndrome)V. S. Dmitruk0V. G. Martynova1S. A. Khardikova2K. A. Skrylova3V. I. Shirshkova4Siberian State Medical UniversitySiberian State Medical UniversitySiberian State Medical UniversitySiberian State Medical UniversitySiberian State Medical UniversityThe Brooke-Spiegler syndrome is a rare autosomal dominant disorder characterized by development of multiple skin tumours including multiple cylindromas and trichoepitheliomas. Here we describe a case of 68-year-old man admitted at a consultative appointment into the surgical department of Siberian State Medical University with numerous asymptomatic, skin-coloured, dense smooth papulonodular lesions from 2 to 5 mm diameter over the face and with multiple pinkish dense smooth dome-shaped nodules from 1 to 5 cm over the scalp. The patient has noted their appearance since childhood. Earlier, similar lesions have been observed in his father and grandmother. Histopathological examination classified facial lesions as trichoepitheliomas and defined scalp lesions as cylindromas, thereby confirming the diagnosis of Brooke-Spiegler syndrome.https://fcm.kemsmu.ru/jour/article/view/869brooke-spiegler syndrometrichoepitheliomacylindroma
spellingShingle V. S. Dmitruk
V. G. Martynova
S. A. Khardikova
K. A. Skrylova
V. I. Shirshkova
Rare Skin Diseases: Turban Tumor (Brooke-Spiegler Syndrome)
Фундаментальная и клиническая медицина
brooke-spiegler syndrome
trichoepithelioma
cylindroma
title Rare Skin Diseases: Turban Tumor (Brooke-Spiegler Syndrome)
title_full Rare Skin Diseases: Turban Tumor (Brooke-Spiegler Syndrome)
title_fullStr Rare Skin Diseases: Turban Tumor (Brooke-Spiegler Syndrome)
title_full_unstemmed Rare Skin Diseases: Turban Tumor (Brooke-Spiegler Syndrome)
title_short Rare Skin Diseases: Turban Tumor (Brooke-Spiegler Syndrome)
title_sort rare skin diseases turban tumor brooke spiegler syndrome
topic brooke-spiegler syndrome
trichoepithelioma
cylindroma
url https://fcm.kemsmu.ru/jour/article/view/869
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AT vgmartynova rareskindiseasesturbantumorbrookespieglersyndrome
AT sakhardikova rareskindiseasesturbantumorbrookespieglersyndrome
AT kaskrylova rareskindiseasesturbantumorbrookespieglersyndrome
AT vishirshkova rareskindiseasesturbantumorbrookespieglersyndrome