AtlasUlcerativeBehçet Disease

Ulcerative Diseases

Behçet Disease

aka Behçet Syndrome · Triple Symptom Complex · Adamantiades-Behçet Disease

Systemic vasculitis with recurrent oral and genital ulcers, uveitis, and multisystem involvement.

Criteria
Oral ulcers + 2 of (genital/eye/skin/pathergy)
HLA
B51
Rx
Steroids, azathioprine, anti-TNF

Red Flags

  • ·Hypopyon
  • ·Sudden vision loss
  • ·Neurological symptoms

§ figuresFigures (2)

Fig. 1Behcets disease Oral Ulcer in patient with the disease.

“Behcets disease” · Ahmet Altiner MD, Rajni Mandal MD · CC BY-SA 3.0 · Wikimedia Commons

Fig. 2Fundus photograph of the case showing optic disc swelling. (A) Right eye, (B) Left eye.

“Behcet fundus” · see above · CC BY 2.0 · Wikimedia Commons

Verified open-access images only, reproduced for education under their stated licences · no AI-generated or illustrative artwork · Behçet Disease figures pending faculty review.

§ overviewOverview

Chronic, relapsing multisystem inflammatory disorder characterised by recurrent oral ulcers, genital ulcers, and ocular inflammation.

§ icdICD Classification

M35.2

§ etiologyEtiology

  • 01Unknown; autoimmune vasculitis
  • 02HLA-B51 association
  • 03Environmental triggers (infectious agents)

§ epidemiologyEpidemiology

Silk Road distribution (Turkey, Middle East, Japan). M = F (M more severe). Peak 20–40 years.

§ pathogenesisPathogenesis

Immune complex-mediated vasculitis → endothelial damage → thrombosis and tissue necrosis.

§ clinicalClinical Features

  • 01Recurrent oral aphthous-like ulcers (major criterion)
  • 02Genital ulcers (scarring)
  • 03Ocular: anterior/posterior uveitis, hypopyon
  • 04Skin: erythema nodosum, acneiform lesions, pathergy
  • 05Neurological, vascular, GI involvement

§ differentialDifferential Diagnosis

  • 01Recurrent aphthous stomatitis
  • 02Herpes simplex
  • 03Pemphigus vulgaris
  • 04Crohn disease
  • 05Reactive arthritis

§ investigationsInvestigations

  • 01Clinical diagnosis (ISG criteria)
  • 02Pathergy test (positive in ~50%)
  • 03HLA-B51
  • 04Ophthalmological exam

§ classificationClassification

  • 01ISG criteria: recurrent oral ulcers (≥3/year) + 2 of: genital ulcers, eye lesions, skin lesions, positive pathergy

§ treatmentTreatment

  • 01Mild: topical steroids, colchicine
  • 02Moderate-severe: systemic steroids, azathioprine, cyclosporine
  • 03Severe: anti-TNF (infliximab), interferon-α
  • 04Anticoagulation if thrombosis

§ complicationsComplications

  • 01Blindness (uveitis)
  • 02CNS disease
  • 03Arterial aneurysms
  • 04DVT/PE

§ prognosisPrognosis

Variable; ocular and CNS involvement worsen prognosis.

§ examKey Examination Points

  • 01Triple symptom complex: oral + genital + ocular
  • 02Pathergy test
  • 03HLA-B51

§ revisionQuick Revision Summary

  • 01Silk Road disease
  • 02Recurrent oral ulcers + genital + uveitis
  • 03Anti-TNF for severe

§ vivaBDS Viva Questions

  • 01What are the ISG criteria?
  • 02What is pathergy?
  • 03Describe the ocular complications.

§ mcqsMCQs — Assessment (3)

Question 1

HLA association with Behçet disease:

Question 2

Pathergy test involves:

Question 3

Eye complication of Behçet:

§ evidenceReferences & Evidence

Current Classification

WHO Classification of Tumours Online — Head and Neck

IARC / WHO · 2022

Continuously updated online edition of the WHO head and neck tumour classification.

View official source

Evidence summary

Highest level of evidence linked to this page: WHO / Official Classification.

  • L1 · Classification — 1
  • L6 · Textbook — 3

1 linked source was published from 2024 onwards. Where guidance is limited or contested, rely on the higher-level sources above rather than this summary. Educational use only — not a substitute for clinical judgement.

Content last reviewed

September 2026

Evidence last reviewed

September 2026

Next scheduled review

September 2027

Review status

EVIDENCE REVIEWED

Search latest PubMed evidence

Last literature search: 7 September 2026

Open PubMed

Current Classification

Reference #1L1 · ClassificationWHO / Classification Verified
WHO Classification of Tumours Online — Head and Neck

IARC / WHO. IARC · 2022

Continuously updated online edition of the WHO head and neck tumour classification.

DOI: Identifier not availablePMID: Identifier not available
View source

Histopathology References

Reference #4L6 · TextbookHistopathology References Unverified
Surgical Pathology of the Head and Neck3rd Edition

Barnes L. CRC Press · 2008

Detailed histopathological reference for head and neck diagnostic criteria.

DOI: Identifier not availablePMID: Identifier not available

Textbook References

Reference #2L6 · TextbookTextbooksRecent Verified
Oral and Maxillofacial Pathology5th Edition

Neville BW, Damm DD, Allen CM et al.. Elsevier · 2024

Standard reference textbook of oral and maxillofacial pathology used throughout this atlas.

DOI: Identifier not availablePMID: Identifier not available
View source
Reference #3L6 · TextbookTextbooks Unverified
Shafer's Textbook of Oral Pathology9th Edition

Rajendran R, Sivapathasundharam B. Elsevier · 2020

Undergraduate and postgraduate oral pathology textbook widely used in BDS and FCPS curricula.

DOI: Identifier not availablePMID: Identifier not available

Additional sources cited on this page

  1. ISG for Behçet's Disease. Lancet 1990;335:1078
  2. Neville BW. Oral & Maxillofacial Pathology, 4e

Browse the full evidence library →

Draft — pending faculty review. Educational use only; verify against current guidelines and primary sources before clinical application.