DISEASE SCANNER

Global Incurable Diseases Tracker

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Rare Disease

Behçet's Disease

HIGH SEVERITY

A rare chronic vasculitis causing recurrent mouth ulcers, genital ulcers, eye inflammation, and skin lesions. Can affect blood vessels of all sizes. Associated with HLA-B51 in some populations. Silk Road disease.

Global Affected

1.0M

Countries

20

Symptoms

Recurrent mouth ulcers
Genital ulcers
Eye inflammation (uveitis)
Skin lesions
Joint pain
Blood vessel inflammation
Brain inflammation
Digestive tract ulcers

Treatment Options

Colchicine
Topical corticosteroids
Azathioprine
Cyclosporine
Anti-TNF agents
Interferon-alpha
Apremilast
Thalidomide
Cyclophosphamide
Corticosteroids

Risk Factors

1Geographic (Silk Road countries)
2Age (20-40)
3Male gender (more severe)
4HLA-B51
5Genetic factors

Diagnostic Methods

  • 1Clinical criteria
  • 2Pathergy test
  • 3HLA-B51 testing
  • 4Eye examination
  • 5Imaging (vascular, CNS)
  • 6Biopsy (rarely)

Prognosis

Variable. Chronic relapsing course. Blindness possible from eye disease. Vascular and neurological complications serious. Males have worse prognosis. Lifelong management. Mortality 5-10% mainly from vascular/CNS disease.

Prevention

  • No known prevention
  • Early treatment
  • Regular monitoring
  • Avoid trauma (pathergy)
  • Smoking cessation

Research Status

Colchicine first-line for mild disease. Azathioprine and cyclosporine for eye involvement. Biologics (anti-TNF, interferon-alpha) for severe disease. Apremilast (PDE4 inhibitor) for oral ulcers. Thalidomide for resistant ulcers.

Sources

  • https://www.mayoclinic.org/diseases-conditions/behcets-disease
  • https://www.hematology.org/education/patients
  • https://www.ncbi.nlm.nih.gov/books
  • https://rarediseases.org/rare-diseases

Medical Disclaimer

This information is for educational purposes only. Always consult healthcare professionals for medical advice, diagnosis, and treatment.