Advertisement

Skin Manifestations of Behçet’s Disease

  • Ayhan DinçEmail author
  • İsmail Şimşek
Chapter

Abstract

Behçet’s disease (BD) is a systemic vasculitis of unknown etiology involving the small and large vessels of both the venous and arterial systems. This disease, first recognized to be a new entity by Turkish dermatologist Hulusi Behçet, was termed for triple symptom complex of oral aphthae, genital ulcers, and hypopyon uveitis until BD became the accepted designation for this disorder. While features constituting the “triple symptom complex” are most commonly encountered among patients with BD, subsequent observations showed that this entity is a multisystem disease characterized by variable clinical manifestations. Mucocutaneous features are the most common and the presenting symptoms of the disease, while eye, vascular, and neurological elements are the most serious. Although mucocutaneous symptoms are not life threatening, they are associated with a substantial decrease in quality of life of BD patients. Skin lesions other than oral and genital ulcers are seen in approximately 80 % of patients with BD. These lesions can be divided into three broad categories: (1) nodular lesions (erythema nodosum-like lesions and superficial thrombophlebitis), (2) papulopustular and acneiform lesions, and (3) other lesions such as skin ulcers and Sweet’s syndrome. In this chapter, important features of mucocutaneous lesion as well as their differential diagnoses were discussed.

Keywords

Genital Herpes Pyoderma Gangrenosum Genital Ulcer Recurrent Aphthous Stomatitis SAPHO Syndrome 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. 1.
    Yurdakul S, et al. Behçet’s syndrome. Best Pract Res Clin Rheumatol. 2008;22(5):793.PubMedCrossRefGoogle Scholar
  2. 2.
    Dinc A, et al. The proportional Venn diagram of Behçet’s disease-related manifestations among young adult men in Turkey. Clin Exp Rheumatol. 2005;23(4 Suppl 38):S86–90.PubMedGoogle Scholar
  3. 3.
    Simsek I, et al. Accuracy of recall of the items included in disease activity forms of Behçet’s disease: comparison of retrospective questionnaires with a daily telephone interview. Clin Rheumatol. 2008;27(10):1255–60.PubMedCrossRefGoogle Scholar
  4. 4.
    Alpsoy E, et al. Mucocutaneous lesions of Behçet’s disease. Yonsei Med J. 2007;48(4):573.PubMedCrossRefGoogle Scholar
  5. 5.
    Keogan MT. Clinical immunology review series: an approach to the patient with recurrent orogenital ulceration, including Behçet’s syndrome. Clin Exp Immunol. 2009;156(1):1.PubMedCrossRefGoogle Scholar
  6. 6.
    Hatemi G, et al. The pustular skin lesions in Behçet’s syndrome are not sterile. Ann Rheum Dis. 2004;63(11):1450.PubMedCrossRefGoogle Scholar
  7. 7.
    Ilknur T, et al. Histopathologic and direct immunofluorescence findings of the papulopustular lesions in Behçet’s disease. Eur J Dermatol. 2006;16(2):146.PubMedGoogle Scholar
  8. 8.
    Demirkesen C, et al. Clinicopathologic evaluation of nodular cutaneous lesions of Behçet syndrome. Am J Clin Pathol. 2001;116:341.PubMedCrossRefGoogle Scholar
  9. 9.
    Varol A, et al. The skin pathergy test: innately useful? Arch Dermatol Res. 2010;302(3):155.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2014

Authors and Affiliations

  1. 1.Rheumatology, Patio ClinicÇankayaTurkey
  2. 2.RheumatologyGulhane School of MedicineAnkaraTurkey

Personalised recommendations