Behçet Syndrome

Behçet's Disease

Overview

Behçet syndrome is a chronic, relapsing, multisystem inflammatory disorder characterized by recurrent oral ulcers, genital ulcers, and ocular inflammation (uveitis). It is classified as a systemic vasculitis and can affect blood vessels of all sizes. The condition is most prevalent along the ancient Silk Road region, from the Mediterranean to East Asia, and is associated with HLA-B51.

Key Symptoms

  • Recurrent painful oral aphthous ulcers (required for diagnosis)
  • Genital ulcers that are painful and may scar
  • Ocular inflammation (uveitis, retinal vasculitis) that can threaten vision
  • Skin lesions including erythema nodosum-like nodules, pseudofolliculitis, and papulopustular lesions
  • Positive pathergy test — a papule or pustule forming at the site of a needle prick
  • Joint pain, blood vessel inflammation, and neurological or gastrointestinal involvement in severe cases

Affected Areas

Oral mucosa, Genital skin, Eyes, Skin (trunk, legs), Joints, Blood vessels, Central nervous system

Care Instructions

  1. See a dermatologist and rheumatologist for multidisciplinary management.
  2. Topical corticosteroids and analgesic mouthwashes for oral ulcers.
  3. Colchicine is often used as a first-line systemic treatment for mucocutaneous disease.
  4. Immunosuppressive agents (azathioprine, mycophenolate) for more severe manifestations.
  5. Biologic agents (anti-TNF therapy, apremilast) for refractory disease.
  6. Regular ophthalmologic exams are critical to prevent vision-threatening complications.

Medical Disclaimer: This information is for educational purposes only and does not replace professional medical advice. Always consult a qualified dermatologist for diagnosis and treatment.

Prevalence: Rare in Western countries; much more common along the Silk Road — prevalence up to 420 per 100,000 in Turkey