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The Mucocutaneous Manifestations and Pathergy Reaction in Behçet Syndrome

  • M. Cem Mat
  • Dongsik Bang
  • Eun-So Lee
  • Zekayi Kutlubay
Chapter

Abstract

The diagnosis of Behçet syndrome (BS) is based on clinical features. There is no specific diagnostic test. Mucocutaneous lesions like recurrent aphthous stomatitis, genital ulcers, and various cutaneous lesions are its foremost manifestations. The presence of erythema nodosum-like lesions, pseudofolliculitis, papulopustular lesions, acneiform nodules, superficial thrombophlebitis, and cutaneous hyperreactivity (pathergy) are included in the criteria of the International Study Group for Behçet’s disease even though there is debate that the presence of some of these lesions in any one patient is coincidental. Mucocutaneous lesions in BS may differ in both clinical and histopathologic appearance during the disease course, and the common underlying pathology is a vasculitis affecting blood vessels of all sizes. Different therapeutic approaches including immunomodulatory or immunosuppressive drugs have been introduced with increasing trend of the newer biological agents.

Keywords

Behçet syndrome Behçet disease Aphthous stomatitis Genital ulcers Erythema nodosum Papulopustular lesions Mucocutaneous lesions Pathergy 

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Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • M. Cem Mat
    • 1
  • Dongsik Bang
    • 2
  • Eun-So Lee
    • 3
  • Zekayi Kutlubay
    • 4
  1. 1.Dermatologist, Private PracticeIstanbulTurkey
  2. 2.Department of DermatologyCatholic Kwandong University International St. Mary’s HospitalIncheonSouth Korea
  3. 3.Department of DermatologyAjou University School of MedicineSuwonSouth Korea
  4. 4.Department of DermatologyIstanbul University, Cerrahpasa Medical Faculty HospitalIstanbulTurkey

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