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

§ 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:

References

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

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