Contributed by: Ritesh Agnihothri, George Washington University School of Medicine and Health Sciences, 2300 Eye Street, NW Washington, D.C. 20037
- Behcet's Disease (BD) refers to a clinical syndrome that affects multiple organ systems and charts a chronic course with exacerbations and remissions. The disease can affect any organ but often comes to clinical manifestation due to its mucocutaneous findings of recurrent oral and genital ulcers as well as other skin lesions.
- BD is most common in the Mediterranean, Middle East, and Asia, although there are sporadic cases throughout the world. Typical age of onset is in the third decade.
|Etiology and Pathogenesis|
- The precise etiopathogenesis of BD is unknown but it is likely an autoimmune or auto-inflammatory disorder occurring in genetically predisposed individuals possibly due to an infectious trigger. There is a strong association with the HLA-B51 allele.
- Although rare, BD should be considered in any individual with recurrent oral and genital ulcers. The disease can involve almost any organ but the mucocutaneous features as well as involvement of the eyes and joints are particularly noteworthy.
- Diagnosis of BD is based on clinical criteria. No diagnostic laboratory test exists.
- Recurrent oral and genital aphthous ulcers
- Recurrent aphthous ulcers of the oral and genital mucosa are often the presenting sign of the disease. These lesions are typically painful and self-resolve.
- Ocular disease
- BD can cause severe inflammation of multiple tissues within the eye and may lead to blindness.
- A non-erosive arthritis is seen in many patients with BD
- Other Organs
- Multiple other organ systems can become inflamed in BD resulting in renal disease, CNS lesions mimicking multiple sclerosis, as well as cardiac, GI, and vascular sequelae.
Sakane, Tsuyoshi, et al. "Behcet's disease." New England Journal of Medicine 341.17 (1999): 1284-1291.
Agnihothri, R, “Behcet Disease” in Pathway Medicine: An Introduction to Clinical Medicine", PathwayMedicine.org (2015).