Behçet's disease
Behçet's disease, also known as Behçet's syndrome or Silk Road disease, is a chronic multisystem condition characterized by recurrent painful ulcers in the mouth and genital areas, as well as inflammation of small blood vessels. Its exact cause remains unknown, but it typically manifests in early adulthood with symptoms that may include skin nodules, joint pain, and, in severe cases, neurological complications such as confusion or paralysis. Importantly, ocular involvement can lead to blindness, making early intervention crucial.
Patients with Behçet's disease may also face an increased risk of blood clots, which can pose serious health risks if they travel to vital areas like the lungs. While the disease has no definitive cure, treatments include topical glucocorticoids, oral steroids, and immunosuppressive agents, which help manage symptoms and flare-ups. Although Behçet's disease can be debilitating, its long-term outlook is generally favorable, with life expectancy similar to that of the general population. The condition has been documented since ancient times, with notable prevalence in regions along the historic Silk Road. Understanding Behçet's disease is essential for improving patient care and outcomes in those affected.
Behçet's disease
ALSO KNOWN AS: Adamantiades-Behçet's disease, Behçet's syndrome, Silk Road disease
ANATOMY OR SYSTEM AFFECTED: Eyes, genitals, mouth, nervous system, skin
DEFINITION: A multisystem disease characterized by recurrent oral and genital ulcers.
CAUSES: Unknown
SYMPTOMS: Inflammation of small blood vessels, oral and genital ulcers, painful skin nodules, joint pain; complications may include mild confusion, paralysis, blindness, blood clots
DURATION: Chronic, with episodes lasting one to two weeks
TREATMENTS: Topical glucocorticoids, thalidomide, oral steroids (prednisone), immunosuppressive agents (azathioprine, cyclosporine)
Causes and Symptoms
Behçet’s disease, or Behçet’s syndrome, usually appears in early adulthood with painful aphthous ulcers (similar to canker sores) in the mouth and on the genitals. These ulcers generally subside after one to two weeks, but they recur throughout the course of the disease. Some patients also have painful skin nodules known as erythema nodosum. Brief periods of joint pain are also common. Rarely, the ulcers of Behçet’s disease are found in the gastrointestinal tract. Involvement of the central nervous system causes a variety of symptoms ranging from mild confusion to paralysis. The most serious complication of Behçet’s disease is involvement of the eyes, since this may progress to blindness. Patients also have an increased predisposition for the formation of blood clots, usually in the venous system. These clots can be life-threatening if they migrate to the lungs.
The common factor underlying these diverse manifestations of Behçet’s disease is inflammation of the small blood vessels, but it remains unclear what environmental or genetic factors trigger this inflammation. Rare reports have been made of familial forms of the disease, raising the possibility of a genetic basis, but this remains under investigation. Diagnosis is made on clinical grounds.
Treatment and Therapy
Behçet’s disease is a chronic condition, but the severity of the disease generally lessens over time. Ulcers are first treated with topical glucocorticoids, in the form of either mouthwash or paste. Apremilast (Otezla) is approved for treatment of oral ulcers. Refractory cases may require thalidomide, a drug that must be used with extreme caution because of its ability to cause birth defects. Because of the risk of blindness, ocular and central nervous system disease is managed more aggressively with oral (such as prednisone), along with azathioprine or cyclosporine.
Apart from the above complications, the long-term of Behçet’s disease is quite good, and the life expectancy of patients is comparable with that of the general population.
Perspective and Prospects
The first description of Behçet’s disease appears in the writings of Hippocrates in the fifth century BCE., but it was first recognized in the modern era by the Turkish physician Hulusi Behçet in 1937. Although Behçet’s disease is seen throughout the world, the highest prevalence is in countries along the ancient Silk Road, a trading route extending from the Far East to the Mediterranean Sea.
Bibliography
"Behçet Disease." Genetics Home Reference, US National Library of Medicine, 1 June 2017, ghr.nlm.nih.gov/condition/behcet-disease. Accessed 28 Mar. 2024.
"Behcet’s Disease." Johns Hopkins Vasculitis Center, Johns Hopkins U / Johns Hopkins Health System, www.hopkinsvasculitis.org/types-vasculitis/behcets-disease/. Accessed 28 Mar. 2023.
"Behçet’s Disease." National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Sept. 2023, www.niams.nih.gov/Health‗Info/Behcets‗Disease/default.asp. Accessed 28 Mar. 2024.
Calamia, Kenneth T. "Medical Summary of Behcet’s Disease." American Behcet’s Disease Association, 1 Aug. 2017, https://www.behcets.com/medical-summary. Accessed 28 July 2024.
Lee, Sungnack, et al., editors. Behçet’s Disease: A Guide to Its Clinical Understanding. Springer, 2001.
Yazici, Yusuf, et al. "Behçet’s Syndrome." Inflammatory Diseases of Blood Vessels, edited by Gary S. Hoffman et al., 2nd ed., Wiley-Blackwell, 2012, pp. 289–98.
Yazici, Yusuf, and Hasan Yazici, editors. Behçet’s Syndrome. Springer, 2010.
Zeis, Joanne. Essential Guide to Behçet’s Disease. Central Vision Press, 2002.