Brook-Spiegler Syndrome Made of Multiple Trichoblastomas only: Clinical and Dermoscopic Appearance
Rasso A*, Oukarfi S, BayBay H, Elloudi S, Mernissi FZ
Department of Dermatology, CHU Hassan II de Fez, Morocco.
*Corresponding Author
Dr. Asmae Rasso,
Department of Dermatology, CHU Hassan II Fez, Morocco.
Tel: 00 212 672314910
E-mail: rassoasmae@gmail.com
Received: March 29, 2019; Accepted : May 06, 2019; Published: May 13, 2019
Citation: Rasso A, Oukarfi S, BayBay H, Elloudi S, Mernissi FZ. Brook-Spiegler Syndrome Made of Multiple Trichoblastomas only: Clinical and Dermoscopic Appearance. Int J Clin Dermatol Res. 2019;7(3):205-206. doi: dx.doi.org/10.19070/2332-2977-1900050
Copyright: Rasso A© 2019. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution and reproduction in any medium, provided the original author and source are credited.
2.Case Report
3.References
Abbreviations
BSS: Brooke-Spiegler Syndrome.
Case Report
Brooke-Spiegler Syndrome (SBS) is a rare, autosomal dominant genodermatosis characterized by the association of multiple benign annexial neoplasms including eccrine spiradenomas, cylindromes, and trichoblastoma [1]. We report the clinical and dermoscopic aspects of a phenotypic variant made of multiple trichoblastomas.
A 42-years-old man who was presented to our department with the clinical complaint of multiple papulonodular lesions on his face evolved since the age of 14 years. His mother also has similar lesions on her face. The man has no clinical symptoms associated with the lesions. At the clinical examination, the patient has numerous papular and nodular of variable size and normal skin color situated at the level of the nasolabial fold, the nose, and the internal nail of the orbit without resulting in any deformation (Figure1, below). Through Dermoscopy, we found out a calcification, an hyperpigmentation and a telangiectasia. (Figure 2 below). The histological study confirms trichoblastoma, and the diagnosis of Brooke-Spiegler Syndrome (SBS) was retained in front of the multiple aspect of lesions.
Figure .: multiple trichoblastomas of a nasolabial fold.
Figure 2. Dermoscopy (A): Calcification; (B): Hyperpigmentation; (C): Telangiectasia.
Trichoblastoma is a benign follicular tumor. Its multiple appearance is reminiscent of Brooke-Speigle syndrome, which is a genodematous mutation of the CYLD tumor suppressor gene located on chromosome 16q12-q13 [1, 3]. The phenotypic mutation is variable even within the same family. Clinically, they can be solitary or multiple and usually occur in adults with an increase in size while the person grows in age. Trichoblastomas are preferentially located in the nasal folds, nose and internal edge of the orbit bilaterally and symmetrical [4]. This syndrome is important to know because would allow us not to mix it with other diseases such as the basal cell carcinomas [2]. Treatment modalities available in patients with SSB include tumor excision, dermabrasion, electrodessication, cryotherapy and radiotherapy with argon and CO2 lasers [5].
This article has presented a family with a phenotypic variant of Brooke-Spiegler syndrome made up of multiple trichoblastomas but without Cylindromas or Spiradenoma. Diagnosing this case was based on clinical dermoscopic and histological criteria.
References
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