Behçet Syndrome: Pathology

  • Cuyan Demirkesen
  • Büge Oz
  • Süha Göksel


Although classified with the vasculitides, a true necrotizing vasculitis is not uniformly observed in many patients with Behçet syndrome (BS). This is especially true for some of the skin mucosa and the central nervous system lesions where nonspecific inflammatory changes rather than a true to form vasculitis are commonly seen. An occlusive vasculopathy is also observed in many patients. A strong association exists between pathological changes in the vasa vasorum and the vascular disease in general.

On the other hand, the vasculitis found can be secondary to chronic inflammation like sometimes seen in the papulopustular lesions and gut involvement. BS has no strict pathognomonic features on histopathology, while it has a rather wide spectrum of microscopic changes. What we know about the histopathology of BS, especially as it relates to vital organs like the heart, eye, brain, and the intestines, we have at hand come from rather small autopsy series, which limits the generalizability of our conclusions.


Histopathology Behçet syndrome Pathology Vasculopathy Vasa vasorum Sweet’s syndrome Mast cells Lymphocytic vasculitis Leukocytoclastic vasculitis Lymphocytic vasculitis Folliculitis Acne vulgaris Comedone Immune complexes Pseudoaneurysm Cardiomegaly Right ventricular thrombi Pulmonary artery aneurysms Endarteritis obliterans Periadventitial fibrosis Nodular opacities in the lung Pleural effusion Parenchymal CNS involvement Non-parenchymal CNS involvement Dural sinus thrombosis Meningoencephalitis Micro-abscess formation Gliosis Brain edema Encephalomyelopathy Gastrointestinal Behçet syndrome Intestinal Behçet syndrome Intestinal ulcers Mesenteric vasculitis Mesenteric vasculopathy Inflammatory bowel disease Crohn’s disease 


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Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Cuyan Demirkesen
    • 1
  • Büge Oz
    • 2
  • Süha Göksel
    • 3
  1. 1.Department of PathologySchool of Medicine, Acıbadem Mehmet Ali Aydınlar UniversityIstanbulTurkey
  2. 2.Department of Pathology, Cerrahpaşa Medical FacultyIstanbul University CerrahpaşaIstanbulTurkey
  3. 3.Pathology LabarotoryAcıbadem Healthcare GroupIstanbulTurkey

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