Skip to main content

Advertisement

Log in

Werner’s syndrome: a quite rare disease for differential diagnosis of scleroderma

  • Case Report
  • Published:
Rheumatology International Aims and scope Submit manuscript

Abstract

Werner’s syndrome (WS) is an autosomal recessive disorder characterized by premature aging. The main features of the disease are scleroderma-like skin appearance, premature atherosclerosis, short stature, diabetes mellitus, early osteoporosis and early aging. Herein, we describe a patient with WS, who has scleroderma-like skin changes and discuss the literature about WS as a disease in the differential diagnosis of systemic sclerosis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3

References

  1. Cohen BA (2008) Other genodermatoses. Premature aging syndromes and pokilodermas. In: Bolognia JL, Jorizzo JL, Rapini RP (eds) Dermatology, 2nd edn. Mosby, St Louis, pp 868–874

    Google Scholar 

  2. Goto M (2002) What can we learn from Werner syndrome? A biased view from a rheumatologist. Mod Rheumatol 12:294–299. doi:10.1007/s101650200052

    Article  CAS  Google Scholar 

  3. Shimamoto A, Sugimoto M, Furuichi Y (2004) Molecular biology of Werner syndrome. Int J Clin Oncol 9:288–298. doi:10.1007/s10147-004-0426-0

    Article  CAS  PubMed  Google Scholar 

  4. Huang S, Lee L, Hanson NB et al (2006) The spectrum of WRN mutations in Werner syndrome patients. Hum Mutat 27:558–567. doi:10.1002/humu.20337

    Article  CAS  PubMed  Google Scholar 

  5. Muftuoglu M, Oshima J, Kobbe CV, Cheng WH, Leistritz DF, Bohr VA (2008) The clinical characteristic of Werner syndrome: molecular and biochemical diagnosis. Hum Genet 124:369–377. doi:10.1007/s00439-008-0562-0

    Article  CAS  PubMed  Google Scholar 

  6. Oppenheimer BS, Kugel VH (1941) Werner’s Syndrome. Am J Med Sci 202:629–642. doi:10.1097/00000441-194111000-00002

    Article  Google Scholar 

  7. Goto M, Takeuchi F, Tanimoto K, Miyamoto T (1985) Clinical, demographic, and genetic aspects of the Werner syndrome in Japan. Adv Exp Med Biol 190:245–261

    CAS  PubMed  Google Scholar 

  8. Chen L, Lee L, Kudlow BA, Dos Santos HG, Sletvold O, Shafeghati Y, Botha EG, Garg A, Hanson NB, Martin GM, Mian IS, Kennedy BK, Oshima J (2003) LMNA mutations in atypical Werner’s syndrome. Lancet 362(9382):440–445. doi:10.1016/S0140-6736(03)14069-X

    Article  CAS  PubMed  Google Scholar 

  9. Goto M (1997) Hierarchical deterioration of body systems in Werner’s syndrome: implication for normal ageing. Mech Ageing Dev 98:239–254. doi:10.1016/S0047-6374(97)00111-5

    Article  CAS  PubMed  Google Scholar 

  10. Ozgenc A, Loeb LA (2006) Werner syndrome, aging and cancer. Genome Dyn 1:206–217. doi:10.1159/000092509

    Article  CAS  PubMed  Google Scholar 

  11. Epstein CJ, Martin GM, Schultz AL, Motulsky AG (1966) Werner’s syndrome: a review of its symptomatology, natural history, pathologic features, genetics and relationship to the natural aging process. Medicine (Baltimore) 45(3):177–221

    CAS  Google Scholar 

  12. Goto M (2000) Werner’s syndrome: from clinics to genetics. Clin Exp Rheumatol 18(6):760–766

    CAS  PubMed  Google Scholar 

  13. Tollefsbol TO, Cohen HJ (1984) Werner’s syndrome: an underdiagnosed disorder resembling premature aging. Age (Omaha) 7:75–88. doi:10.1007/BF02432205

    Article  Google Scholar 

  14. Hettema ME, Zhang D, de Leeuw K, Stienstra Y, Smit AJ, Kallenberg CG, Bootsma H (2008) Early atherosclerosis in systemic sclerosis and its relation to disease or traditional risk factors. Arthritis Res Ther 10(2):R49. doi:10.1186/ar2408

    Article  PubMed  Google Scholar 

  15. Hinchcliff M, Varga J (2008) Systemic sclerosis/scleroderma: a treatable multisystem disease. Am Fam Physician 78(8):961–968

    PubMed  Google Scholar 

  16. Korn JH, Mayes M, Matucci Cerinic M, Rainisio M, Pope J, Hachulla E et al (2004) Digital ulcers in systemic sclerosis: prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rheum 50:3985–3993. doi:10.1002/art.20676

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Cemal Bes.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Bes, C., Vardı, Ş., Güven, M. et al. Werner’s syndrome: a quite rare disease for differential diagnosis of scleroderma. Rheumatol Int 30, 695–698 (2010). https://doi.org/10.1007/s00296-009-0982-8

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00296-009-0982-8

Keywords

Navigation