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Multiple familiäre Trichoepitheliome (Brooke-Syndrom)

Eine seltene Genodermatose mit möglicher periokulärer Manifestation

Multiple familiar trichoepithelioma (Brooke’s syndrome)

A rare autosomal dermatosis with possible periocular manifestation

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Zusammenfassung

Das autosomal-dominant vererbte Brooke-Syndrom ist durch das Auftreten multipler Trichoepitheliome, die als dichtstehende Knötchen im Gesichtsbereich imponieren, gekennzeichnet. Die Therapie besteht bei größeren Tumoren in der Exzision, bei flächenhaft ausgebreiteten Tumoren in der Dermabrasion oder Laserabtragung. Patienten mit Brooke-Syndrom sollten regelmäßig kontrolliert werden, da maligne Hauttumoren auftreten können. Wir stellen hier einen Patienten mit einem Brooke-Syndrom vor und berichten über den Behandlungsverlauf.

Abstract

Brooke’s syndrome is a rare autosomal dominant dermatosis characterized by multiple trichoepitheliomas, which preferentially arise in the face. Therapy consists of excisional surgery of larger tumors and for multiple lesions, dermal abrasion or laser therapy may be considered. Patients with Brooke’s syndrome should be closely followed-up due to the possible development of malignant skin tumours. Here, we present a patient with Brooke’s syndrome and report on the course of treatment.

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Literatur

  1. Bowen S, Gill M, Lee DA et al. (2005) Mutations in the CYLD gene in Brooke-Spiegler Syndrome, familial cylindromatosis and multiple familial trichoepithelioma: lack of genotype-phenotype correlation. J Invest Dermatol 124: 919–920

    Article  PubMed  CAS  Google Scholar 

  2. Delfino M, D’Anna F, Ianniello S, Donofrio V (1991) Multiple hereditary trichoepithelioma and cylindroma (Brooke-Spiegler syndrome). Dermatologica 183: 150–153

    Article  PubMed  CAS  Google Scholar 

  3. Gottschalk HR (1974) Proceedings: Dermal eccrine cylindroma, epithelioma adenoides cysticum of Brooke, and ecrine spiradenoma. Arch Dermatol 110: 473–474

    Article  PubMed  CAS  Google Scholar 

  4. Hu G, Onder M, Gill M et al. (2003) A novel missense mutation in CYLD in a family with Brooke-Spiegler syndrome. J Invest Dermatol 121: 732–734

    Article  PubMed  CAS  Google Scholar 

  5. Korting HC, Konz B (1982) Coincidence of multiple cylindroma and trichoepitheliomas (Brooke-Spiegler-Syndrome) Article in German. Hautarzt 33: 34–46

    PubMed  CAS  Google Scholar 

  6. Lee KH, Kim JE, Cho BK et al. (2008) Malignant transformation of multiple trichoepithelioma: case report and literature review. Acta Derm Venerol 88: 43–46

    Article  PubMed  Google Scholar 

  7. Martins C, Bártolo E (2000) Brooke-Spiegler syndrome: treatment of cylindromas with CO2 laser. Dermatol Surg 26: 877–880

    Article  PubMed  CAS  Google Scholar 

  8. Osborne JP (1988) Diagnosis of tuberous sclerosis. Arch Dis Child 63: 1423–1425

    Article  PubMed  CAS  Google Scholar 

  9. Pizinger K, Michal M (2000) Malignant cylindroma in Brooke-Spiegler syndrome. Dermatology 201: 255–257

    Article  PubMed  CAS  Google Scholar 

  10. Rallan D, Harland CC (2005) Brooke-Spiegler syndrome: treatment with laser ablation. Clin Exp Dermatol 30: 355–357

    Article  PubMed  CAS  Google Scholar 

  11. Retamar RA, Stengel F, Saadi ME et al. (2007) Brooke-Spiegler syndrome – report of four families: treatment with CO2 laser. Int J Dermatol 46: 583–586

    Article  PubMed  Google Scholar 

  12. Saunders H, Tucker P, Saurine T, Watkins F (2003) Pedigree of multiple benign adnexal tumours of Brooke-Spiegler type. Australas J Dermatol 44: 144–148

    Article  PubMed  Google Scholar 

  13. Scheinfeld N, Hu G, Gill M et al. (2003) Identification of a recurrent mutation in the CYLD gene in Brooke-Spiegler syndrome. Clin Exp Dermatol 28: 539–541

    Article  PubMed  CAS  Google Scholar 

  14. Schirren CG, Wörle B, Kind P, Plewig G (1995) A nevoid plaque with histological changes of trichoepithelioma and cylindroma in Brooke-Spiegler syndrome. An immunohistochemical study with cytokeratins. J Cutan Pathol 22: 563–569

    Article  PubMed  CAS  Google Scholar 

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Correspondence to F. Schlichtenbrede.

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Rensch, F., Harder, B., Goebeler, M. et al. Multiple familiäre Trichoepitheliome (Brooke-Syndrom). Ophthalmologe 106, 735–739 (2009). https://doi.org/10.1007/s00347-008-1848-2

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  • DOI: https://doi.org/10.1007/s00347-008-1848-2

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