Behçet's Disease: The Rare "Silk Road" Condition Behind Recurring Mouth and Genital Sores — and How Treatment Keeps Most People Living Full Lives
Updated: 2 days ago
Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: October 2026
Quick answer
Behçet's disease is a rare, chronic inflammatory disease caused by blood vessel inflammation that damages arteries and veins, also called Silk Road disease [1]. It causes recurring mouth sores in almost all patients, plus genital sores, eye inflammation and joint pain [1]. There is no cure, but corticosteroids and other drugs manage symptoms, and most patients live full lives [1].
What the research says
According to PubMed:
Apremilast reduced mouth ulcers in a phase 3 trial. In 207 patients with Behçet's syndrome and active oral ulcers but no major organ involvement (104 on apremilast 30 mg twice daily, 103 on placebo), the area under the curve for the number of oral ulcers over 12 weeks was 129.5 with apremilast versus 222.1 with placebo (difference −92.6; 95% CI −130.6 to −54.6; P<0.001). Adverse events included diarrhea, nausea, and headache. (Hatemi et al., N Engl J Med, 2019) [2].
European guidelines tie treatment to which organ is involved. The 2018 EULAR update, written by a task force of experts in internal medicine, rheumatology, ophthalmology, dermatology, neurology, gastroenterology, oral health medicine and vascular surgery, plus two patients, revised the recommendations for skin and mouth, joint, eye, vascular, neurological and gastrointestinal involvement and added a recommendation on surgery for vascular disease. (Hatemi et al., Ann Rheum Dis, 2018) [3].
Newer options are emerging, but strong trials are still limited. A review reported that apremilast was approved in the United States for oral ulcers of Behçet syndrome, that long-term use of tumor necrosis factor inhibitors, especially for eye disease, appears safe and effective, and that ustekinumab, secukinumab and tocilizumab have early promising data but need more study. The author noted a lack of placebo-controlled randomized trials for most treatments. (Yazici, Curr Opin Rheumatol, 2020) [4].
Study details are from PubMed abstracts.
What is Behçet's disease?
Behçet's disease, also known as Silk Road disease, is a chronic (long-term) inflammatory condition caused by vasculitis — inflammation of the blood vessels. This inflammation affects and damages both your arteries and your veins [1].
The condition can create issues in your mouth and genital area in the form of recurrent sores. It is mostly associated with inflammation of both the blood vessels and the eyes, though it often causes many different issues throughout the body.
How common is Behçet's disease?
Behçet's disease is seen all over the world, but it is far more common in some regions than others. It is most common in Northern Turkey (up to 420 cases per 100,000 people), the Mediterranean basin and Middle East (up to 300 cases per 100,000 people), and the Far East (about 15 cases per 100,000 people). It is not as common in the United States (about 7 cases per 100,000 people [1]).
Anyone can develop this disease at any age, though symptoms usually begin to appear between the ages of 20 and 30 [1]. Men and women are equally affected overall.
Region | Cases per 100,000 people |
Northern Turkey | Up to 420 |
Mediterranean basin and Middle East | Up to 300 |
Far East | About 15 |
United States | About 7 |
What are the symptoms of Behçet's disease?
The main symptoms include mouth sores and/or genital sores that keep coming back, skin and joint pain, and inflammation in the eyes [1]. The brain, nerves, lungs (rarely), intestinal tract, and kidneys can also be affected.
The disease affects everybody differently. Some of the most commonly affected areas and their key features are described below.
Body area | Key features |
Mouth | Sores in all patients — recurrent, painful, look like canker sores but more numerous, frequent, and painful; on the lips, tongue, and inside the cheek |
Genitals | Sores that look similar to mouth sores and may be painful; on the scrotum in men and the vulva in women |
Eyes | Pain, blurry vision, light sensitivity, tears, redness; may eventually lead to vision loss |
Skin | Acne-like spots, tender coin-shaped nodules (erythema nodosum), shallow or deep painful ulcers; red bump after scratching (positive pathergy test) |
Joints | Pain in ankles, knees, elbows, and hips; swelling, redness, tenderness; usually no permanent damage |
Veins | Clots and blockages, or a vein closing completely; can affect the vena cava — serious health problems |
Brain | Fever, headache, stiff neck, trouble coordinating movement; stroke possible |
Digestive tract | Abdominal pain or blood in stool from mouth- and genital-style lesions; intestine can bleed and/or rupture |
Other organs | Lungs (rare), kidneys, and large vessels like the aorta occasionally affected |
Why do the mouth sores keep coming back?
Mouth sores occur at some time in all patients. They are usually recurrent — keep coming back — and painful, affecting almost all patients [1] with Behçet's disease. The sores look like the common canker sore but are more numerous, frequent, and painful. They are often the first symptom that a person notices and may occur long before any other symptoms appear. Mouth ulcers are seen on the lips, tongue, and inside the cheek [1].
What do the genital sores look like?
Genital sores look similar to mouth sores and may be painful. They are not as common as mouth sores. They appear on the scrotum (the sack enclosing the testes) in men and on the vulva (the external genital organs) in women [1].
How does Behçet's disease affect the eyes?
Eye inflammation can cause pain, blurry vision, light sensitivity, tears, or eye redness. Behçet's disease may eventually lead to vision loss. Severe eye disease that can cause blindness is more common in the Middle East and Japan than in the United States [1].
What is a positive pathergy test?
Skin problems are a common symptom. They may look like acne or tender, coin-shaped nodules called erythema nodosum, or ulcers that may be shallow or deep and painful. A red bump or sore may develop if the skin is scratched or pricked. Doctors call this a positive pathergy test [1].
Why does the vein inflammation matter?
Vein inflammation can lead to clots and blockages or cause a vein to close completely. It can affect both superficial veins (those close to the surface of the skin) and deep veins. It may also affect the largest vein in the body (the vena cava), which could lead to serious health problems. These vein problems are caused by inflammation, not a flaw in the body [1]'s clotting system.
How serious can it get?
The brain, particularly the meninges (the brain's covering), can also be affected. Symptoms of inflammation in the brain include fever, headache, a stiff neck, and difficulty coordinating movement [1]. A stroke is also possible, which occurs when blood vessels in the brain are either blocked or ruptured.
Gastrointestinal tract features include abdominal pain or blood in your stool, caused by lesions similar to those seen in the mouth and genital area. Lesions in the GI tract are more dangerous because they can cause the intestine to bleed and/or rupture [1]. Other organs, like the lungs (rarely) or kidneys, and large vessels such as the aorta, can occasionally be affected.
What causes Behçet's disease?
The exact cause of Behçet's disease is unknown, though genes, infections and the immune system may all play a role. It may be an autoimmune disorder, meaning the body's immune system mistakenly attacks healthy tissue with an inflammatory response [1].
Both HLA-B5 and HLA-B51 are gene markers that are sometimes present in patients [1] with Behçet's disease. But some people who have this gene marker don't have the disease. Researchers are studying other genes related to immune function and think that infections — either bacteria or a virus — may play a role in triggering the disease in some people who have genetic markers that predispose them to Behçet's.
Genetic and environmental factors are both likely factors in the development of Behçet's disease.
Possible cause factor | What the source says |
Autoimmune response | The immune system may mistakenly attack healthy tissue with an inflammatory response |
Gene markers | HLA-B5 and HLA-B51 are sometimes present in patients, but not everyone with the marker gets the disease |
Infections | Bacteria or a virus may trigger the disease in people with predisposing genetic markers |
Overall | Genetic and environmental factors are both likely involved |
Who is at risk?
There are a few different groups of people that are more likely to develop this disease than others, including those who live in the parts of the world where this disease is the most common, people aged 20 to 40, those with specific genes (since HLA-B5 or HLA-B51 are present in some patients), and men, who are more at risk of developing this disease than women [1].
Is Behçet's disease hereditary?
There's no clear evidence to suggest Behçet's disease is an inherited disease. Most cases develop randomly without any family link. While it has been reported that a small percentage of cases occur in the same family, there is no clear inheritance pattern [1].
How is Behçet's disease diagnosed?
No single laboratory test can diagnose Behçet's disease, so doctors rely on the pattern of symptoms and rule out similar illnesses. The diagnosis is usually made based on your symptoms, including how often — typically at least three times a year [1] — oral ulcers come back, plus at least two more of the following:
Diagnostic criterion | Details |
Recurring oral ulcers | At least three times a year |
Genital sores | Sores similar to the mouth sores |
Eye inflammation | Pain, redness, blurry vision, light sensitivity |
Skin problems | Acne-like spots, nodules, ulcers |
Positive pathergy test | A red bump develops a few days after the skin is pricked |
To make the correct diagnosis, other illnesses that produce mouth sores and closely resemble Behçet's disease need to be ruled out. Your doctor may order a blood test, which can help rule out some of these other conditions, including systemic lupus, Crohn's disease (an intestinal inflammatory condition), and other forms of vasculitis [1].
How is Behçet's disease treated?
Behçet's disease has no known cure, but drugs such as corticosteroids, colchicine, other immunosuppressants and biologics can help manage its symptoms.
Medication | Role in treatment |
Corticosteroids (e.g., prednisone) | The main treatment — suppress inflammation and immune function |
Colchicine (Colcrys) | Helps with mouth sores, genital sores, and possibly joint pain |
Immunosuppressants (methotrexate, azathioprine, cyclophosphamide, cyclosporine) | Control immune overactivity in more involved disease |
Biologics (anti-TNF, infliximab, etanercept, tocilizumab) | Used in more severe cases; raise susceptibility to infections |
Apremilast (Otezla) | Oral medication approved for treating mouth ulcers in Behçet's disease |
Corticosteroids, such as prednisone, are the main treatment [1]. These drugs suppress inflammation and immune function.
Colchicine (Colcrys) can help with mouth sores, genital sores, and possibly joint pain [1].
Other immunosuppressant drugs such as methotrexate (Trexall, Rasuvo), azathioprine (Imuran, Azasan), cyclophosphamide (Cytoxan, Neosar), and cyclosporine (Gengraf, Neoral, Sandimmune) are used. Also, biologics such as anti-TNF (Tumor Necrosis Factor), infliximab (Remicade), etanercept (Enbrel), and others such as tocilizumab (Actemra) are used in more severe cases. Since these are immunosuppressant drugs, they can make you more susceptible to other infections [1].
Apremilast (Otezla), an oral medication taken by mouth, is approved for treating mouth ulcers [1] [4] in patients with Behçet's disease.
Depending on the severity of your condition, your doctor may also suggest using certain eye drops, mouthwashes, and skin ointments [1] to control this disease and its symptoms before deciding to use medication.
What's the prognosis?
Behçet's is a chronic (long-term) disease that can disappear and reappear, no matter the treatment. This can make it difficult to tell how well treatment is working. Most patients with Behçet's will likely deal with symptoms, on and off, for their entire lives. But most are able to live a full life [1].
Is Behçet's disease fatal?
Death occurs in about 5% of patients [1]. Causes of death are most often intestinal perforation (a hole), strokes, and a rupture of enlarged, weakened blood vessels (aneurysms) [1].
How can people cope with Behçet's disease?
Both rest and activity are helpful coping mechanisms. A consistent exercise regimen will make your body feel better, easing the pain in your joints and other problem areas [1]. Alternatively, taking some time to rest when you begin to feel harsh pain will also improve your overall mood and lessen the effect of some symptoms.
Another way to cope is through shared experience and community — finding others who also have this condition. This can sometimes be difficult due to the rarity of this disease, but your doctor can help you find others who may be having similar experiences.
What's the bottom line?
Behçet's disease is a rare, chronic inflammatory condition of the blood vessels that keeps coming back — no matter the treatment — with its signature recurring mouth and genital sores, eye inflammation that can threaten vision, and skin, joint, vein, and organ involvement. There is no cure, but the toolbox is real: corticosteroids as the main treatment, colchicine, immunosuppressants, biologics for severe cases, an approved oral medication for mouth ulcers, and simple eye drops, mouthwashes, and ointments for milder symptoms. Most patients deal with on-and-off symptoms for their entire lives yet still live full lives — especially when they pair consistent exercise with rest, work closely with their doctor, and connect with others who share the experience. Because vein clots, GI bleeding, and brain inflammation can be serious, new or worsening symptoms should always be checked by a provider promptly.
Conclusion and what to do next
Dealing with recurring sores or unexplained inflammation? Talk with your healthcare provider about the pattern of your symptoms, how often they return, and whether your eyes, joints, or digestive tract are involved. Explore Rinnit.com's library of clear, practical health guides to understand your symptoms and start the right conversation with your provider.
Frequently asked questions
What is Behçet's disease?
Behçet's disease (also called Silk Road disease) is a chronic inflammatory condition caused by vasculitis — inflammation of the blood vessels — that damages both arteries and veins and can affect the mouth, genitals, eyes, skin, joints, brain, and digestive tract.
Why is it called "Silk Road disease"?
Because it is most common along the historic Silk Road region — up to 420 cases per 100,000 people in Northern Turkey, up to 300 per 100,000 in the Mediterranean basin and Middle East, and about 15 per 100,000 in the Far East — while it affects only about 7 per 100,000 in the United States.
What are the main symptoms?
The main symptoms are mouth sores and/or genital sores that keep coming back, skin and joint pain, and inflammation in the eyes.
What do the mouth sores look like?
They look like common canker sores but are more numerous, frequent, and painful, appearing on the lips, tongue, and inside the cheek. They occur at some time in all patients and are often the first symptom noticed.
Can Behçet's disease cause blindness?
Eye inflammation can cause pain, blurry vision, light sensitivity, tears, or redness and may eventually lead to vision loss. Severe eye disease that can cause blindness is more common in the Middle East and Japan than in the United States.
What is a positive pathergy test?
A test where the skin is pricked and a red bump or sore develops a few days later — a sign of the disease.
What causes Behçet's disease?
Scientists are not exactly sure. It may be an autoimmune disorder involving gene markers HLA-B5 and HLA-B51 plus environmental triggers such as bacterial or viral infections.
Who is at higher risk?
People who live where the disease is most common, those aged 20–40, people carrying the HLA-B5 or HLA-B51 gene markers, and men (who are more at risk than women, per the source).
Is it inherited?
There's no clear evidence it is inherited. Most cases develop randomly without any family link, and while a small percentage occur in the same family, there is no clear inheritance pattern.
How is it diagnosed?
There is no single laboratory test. Diagnosis is based on oral ulcers recurring at least three times a year plus at least two of the following: genital sores, eye inflammation, skin problems, or a positive pathergy test. Blood tests help rule out conditions like lupus, Crohn's disease, and other vasculitides.
Is there a cure?
No. There is no known cure, but several medications manage symptoms.
What medications treat Behçet's disease?
Corticosteroids (like prednisone) are the main treatment; colchicine helps sores and possibly joint pain; immunosuppressants and biologics are used in more severe cases; and apremilast (Otezla) is approved for mouth ulcers. Eye drops, mouthwashes, and skin ointments may be tried first depending on severity.
Will I have symptoms for life?
Most patients deal with symptoms on and off for their entire lives, since the disease can disappear and reappear no matter the treatment — but most are able to live a full life.
Is Behçet's disease fatal?
Death occurs in about 5% of patients, most often from intestinal perforation, strokes, or rupture of enlarged, weakened blood vessels (aneurysms).
How can I cope with Behçet's disease?
Both rest and activity help — a consistent exercise regimen eases joint and other pain, and resting during harsh pain improves mood and lessens symptoms. Connecting with others who have the condition also helps, and your doctor can help you find them.
Related reading
Uveitis Symptoms, Causes and Treatment: What Eye Inflammation Means for Your Vision — how inflammation inside the eye is evaluated and managed
Vasculitis: Symptoms, Causes and Treatment — the family of conditions behind Behçet's blood vessel damage
The information in this article is for general informational purposes only and is not intended as professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read here.
References
Cleveland Clinic. Behcet's Disease. Cleveland Clinic. Updated June 2, 2020. https://my.clevelandclinic.org/health/diseases/12980-behcets-disease
Hatemi G, et al. Trial of Apremilast for Oral Ulcers in Behçet's Syndrome. N Engl J Med. 2019;381(20):1918-1928. PMID 31722152. https://doi.org/10.1056/NEJMoa1816594
Hatemi G, et al. 2018 update of the EULAR recommendations for the management of Behçet's syndrome. Ann Rheum Dis. 2018;77(6):808-818. PMID 29625968. https://doi.org/10.1136/annrheumdis-2018-213225
Yazici Y. Management of Behçet syndrome. Curr Opin Rheumatol. 2020;32(1):35-40. PMID 31609785. https://doi.org/10.1097/BOR.0000000000000666
National Library of Medicine. Behçet disease. MedlinePlus Genetics. Updated June 1, 2017. https://medlineplus.gov/genetics/condition/behcet-disease/
Calamia K. Medical Summary of Behcet's Disease. American Behcet's Disease Association. Revised August 1, 2017. https://www.behcets.com/medical-summary
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