Behcet's disease Flashcards
What is Behçet’s disease?
A complex inflammatory condition affecting the blood vessels and tissues, characterized by recurrent oral and genital ulcers, and it can affect multiple organs such as the eyes, skin, gastrointestinal tract, and central nervous system.
What gene is associated with Behçet’s disease?
HLA B51 gene.
What are common differential diagnoses for mouth ulcers in Behçet’s disease?
- Simple aphthous ulcers
- Squamous cell carcinoma
- Herpes simplex virus
- Hand, foot, and mouth disease
- Inflammatory bowel disease (e.g., Crohn’s disease)
- Coeliac disease
- Connective tissue diseases (e.g., rheumatoid arthritis, SLE)
- Vitamin deficiencies (e.g., iron, B12, folate)
- HIV
What is the appearance of oral ulcers in Behçet’s disease?
Painful, sharply circumscribed erosions with a red halo that heal over 2-4 weeks. They occur on the oral mucosa and can recur at least 3 times per year.
What are genital ulcers like in Behçet’s disease?
Similar in appearance to oral ulcers, and may leave a scar after healing.
Which organs can be affected by Behçet’s disease?
- Skin: Erythema nodosum, acne-like papules, pustules
- Eyes: Uveitis, retinal vasculitis, thrombosis
- Musculoskeletal: Stiffness, arthralgia, oligoarthritis
- Gastrointestinal: Ulcers in the ileum, caecum, and ascending colon
- CNS: Aseptic meningitis, cerebral venous sinus thrombosis
- Arteries: Aneurysms
- Veins: Deep vein thrombosis, Budd-Chiari syndrome
What is the Pathergy test used for?
To test for non-specific skin hypersensitivity. A positive result shows erythema and induration after multiple needle pricks, which can indicate Behçet’s disease.
What is the first-line management for oral ulcers in Behçet’s disease?
Topical steroids (e.g., soluble betamethasone tablets) and systemic steroids (e.g., oral prednisolone).
What treatment is useful for genital ulcers in Behçet’s disease?
Topical anaesthetics like lidocaine ointment.
What systemic treatment options are available for Behçet’s disease?
- Colchicine (for symptoms)
- Immunosuppressants (e.g., azathioprine)
- Biologic therapy (e.g., infliximab)
What is the prognosis for Behçet’s disease?
It follows a relapsing-remitting course. Most patients have a normal life expectancy, but major complications include vision loss, ruptured pulmonary aneurysms, bowel perforation, and neuro-Behçet’s disease.
What are some complications of Behçet’s disease?
- Vision loss (due to ocular involvement)
- Pulmonary aneurysms (can rupture)
- Bowel perforation
- Neuro-Behçet’s disease (CNS involvement)
