Skip to main content

Advertisement

SpringerLink
Log in

Digital ischemia in Behçet’s disease: case-based review

  • Case Based Review
  • Published:
Rheumatology International Aims and scope Submit manuscript

Abstract

Behçet’s disease is a rare chronic autoimmune disease affecting primarily Middle and East Asian populations between the ages of 20 and 40 years. Behçet’s disease manifests with oral and genital mucocutaneous lesions, ocular disease, venous thrombosis, and central nervous system degradation. Treatment can be challenging and may require immunosuppressive agents and/or topical wound-care. While larger vascular involvement has been reported, digital ischemia due to small-vessel involvement has not been well described in the literature. Based on a systematic literature review, we were only able to find seven published cases of limb ischemia, none of which reported digital involvement. We present a unique case of Behçet’s disease with severe digital ischemia and ulceration caused by small-vessel involvement. The patient was managed successfully with antiplatelet, immunosuppressants, and anti-inflammatories with complete resolution of the ischemic symptoms. By focusing on small-vessel involvement and digital ischemia, we provide insight into clinical presentation and treatment for this very rare vascular manifestation of Behçet’s disease.

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

Access this article

Log in via an institution

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

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Yurdakul S, Hamuryudan V, Yazici H (2004) Behçet syndrome. Curr Opin Rheumatol 16:38–42

    Article  Google Scholar 

  2. Neves FS, Caldas CA, Lage LV, Goldenstein-schainberg C, Gonçalves CR (2009) Faraway from the silk route: demographic and clinical features of Behçet’s disease in 106 Brazilian patients. Clin Rheumatol 28:543–546

    Article  Google Scholar 

  3. Calamia KT, Wilson FC, Icen M, Crowson CS, Gabriel SE, Kremers HM (2009) Epidemiology and clinical characteristics of Behçet’s disease in the US: a population-based study. Arthritis Rheum 61:600–604

    Article  Google Scholar 

  4. Davatchi F, Shahram F, Chams-davatchi C et al (2012) Behcet’s disease: is there a gender influence on clinical manifestations? Int J Rheum Dis 15:306–314

    Article  Google Scholar 

  5. Poddighe D, Mukusheva Z, Dauyey K, Assylbekova M (2019) Adalimumab in the treatment of pediatric Behçet’s disease: case-based review. Rheumatol Int 39:1107–1112

    Article  CAS  Google Scholar 

  6. Zaide N, Awada H (2006) Late onset Behcet’s disease. Joint Bone Spine 73:567–569

    Article  Google Scholar 

  7. Rizzi R, Bruno S, Dammacco R (1997) Behçet’s disease: an immune-mediated vasculitis involving vessels of all sizes. Int J Clin Lab Res 27:225–232

    Article  CAS  Google Scholar 

  8. Direskeneli H (2001) Behçet’s disease: infectious aetiology, new autoantigens, and HLA-B51. Ann Rheum Dis 60:996–1002

    Article  CAS  Google Scholar 

  9. Esatoglu SN, Kutlubay Z, Ucar D et al (2017) Behçet’s syndrome: providing integrated care. J Multidiscip Healthc 10:309–319

    Article  Google Scholar 

  10. Lee ES, Bang D, Lee S (1997) Dermatologic manifestation of Behçet’s disease. Yonsei Med J 38:380–389

    Article  CAS  Google Scholar 

  11. Tugal-tutkun I, Onal S, Altan-yaycioglu R, Huseyin-altunbas H, Urgancioglu M (2004) Uveitis in Behçet disease: an analysis of 880 patients. Am J Ophthalmol 138:373–380

    Article  Google Scholar 

  12. Kötter I, Eckstein AK, Stübiger N, Zierhut M (1998) Treatment of ocular symptoms of Behçet’s disease with interferon alpha 2a: a pilot study. Br J Ophthalmol 82:488–494

    Article  Google Scholar 

  13. Hatemi G, Christensen R, Bang D et al (2018) 2018 update of the EULAR recommendations for the management of Behçet’s syndrome. Ann Rheum Dis 77:808–818

    PubMed  Google Scholar 

  14. Erkan F, Gül A, Tasali E (2001) Pulmonary manifestations of Behçet’s disease. Thorax 56:572–578

    Article  CAS  Google Scholar 

  15. Seyahi E, Yurdakul S (2011) Behçet’s syndrome and thrombosis. Mediterr J Hematol Infect Dis 3:e2011026

    Article  Google Scholar 

  16. Alibaz-oner F, Karadeniz A, Ylmaz S et al (2015) Behçet disease with vascular involvement: effects of different therapeutic regimens on the incidence of new relapses. Medicine (Baltimore) 94:e494

    Article  CAS  Google Scholar 

  17. Seyahi E, Karaaslan H, Ugurlu S, Yazici H (2013) Fever in Behçet’s syndrome. Clin Exp Rheumatol 31:64–67

    PubMed  Google Scholar 

  18. ITR-ICBD (2014) The International Criteria for Behçet’s Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol 28:338–347

    Article  Google Scholar 

  19. Saip S, Akman-demir G, Siva A (2014) Neuro-Behçet syndrome. Handb Clin Neurol 121:1703–1723

    Article  Google Scholar 

  20. Mat C, Yurdakul S, Sevim A, Özyazgan Y, Tüzün Y (2013) Behçet’s syndrome: facts and controversies. Clin Dermatol 31:352–361

    Article  Google Scholar 

  21. Saadoun D, Wechsler B, Desseaux K et al (2010) Mortality in Behcet’s disease. Arthritis Rheum 62:2806–2812

    Article  CAS  Google Scholar 

  22. Elsharawy MA, Hassan KA, Al-awami M, Al-mulhim FA (2004) Dramatic vascular course of Behcet’s disease. Saudi Med J 25:2013–2015

    PubMed  Google Scholar 

  23. Iscan ZH, Vural KM, Bayazit M (2005) Compelling nature of arterial manifestations in Behcet disease. J Vasc Surg 41:53–58

    Article  Google Scholar 

  24. Akiyama K, Hirota J, Ohkado A, Shiina Y (1998) Multivarious clinical manifestations of multiple pseudoaneurysms in Behçet’s disease. J Cardiovasc Surg (Torino) 39:175–178

    CAS  Google Scholar 

  25. Sechas MN, Liapis CD, Gougoulakis AG, Mandrekas DP, Fotiadis CI, Vaiopoulos GA (1989) Vascular manifestations of Behçet’s disease. Int Angiol 8:145–150

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Minneapolis Heart Institute®, Abbott Northwestern Hospital part of Allina Health, 33300, 920 E. 28th Street, Suite 300, Minneapolis, MN, 55407, USA

    Gopika Hari & Nedaa Skeik

Authors
  1. Gopika Hari
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Nedaa Skeik
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

All listed co-authors have contributed toward case design and data collection and interpretation. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work in accordance with ICMJE 4 authorship recommendation.

Corresponding author

Correspondence to Nedaa Skeik.

Ethics declarations

Conflict of interest

The authors declare that there is no conflict of interest or financial disclosures.

Informed consent

Informed consent was obtained from the individual patient included in the study.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Hari, G., Skeik, N. Digital ischemia in Behçet’s disease: case-based review. Rheumatol Int 40, 137–143 (2020). https://doi.org/10.1007/s00296-019-04452-z

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00296-019-04452-z

Keywords

Search

Navigation