Behçet Disease
- Systemic vascular inflammatory disorder affecting skin, mucous membranes, eyes, lungs, GI tract and CNS. It is characterized by:
- Mouth sores- aphthous ulcers usually first sign
- Genital sores- (75%) small ulcers on scrotum or labia
- Erythema Nodosum- painful red skin nodules
- Also puss filled bumps in the skin
- Arthritis
- Typically symptoms start in the 20's or 30's
Ocular Findings
- Ocular Inflammation (66%)
- Conjunctivitis
- Uveitis
- Hypopion
- Panuveitis with vasculitis
- Complications of uveitis
- cataracts, synechiae, band keratopathy
Other More Rare Findings
- Inflammation of the brain or spinal cord
- blood clots, aneurism, GI vasculitis
Etiology
- Unknown autoimmune disorder
- Associated with the HLA-B51/B5 allele
- 57% of people with this genotype have Behçet syndrome