What's Behçet disease?
Behçet disease is a rare, long-term inflammatory condition of the blood vessels (a vasculitis) that typically causes recurring mouth and genital ulcers, eye inflammation, and skin lesions, and can affect many parts of the body.
| Also indexed as | ORPHA:117, MONDO:0007191 |
|---|---|
| Features mapped | 13 |
| Treatments mapped | 6 |
| Published sources | 15 |
| Last reviewed | 2026-08-04 |
Signs and symptoms
Headache
A post-dural puncture headache is a common complication of spinal or epidural anesthesia. It can slow recovery after surgery and significantly affect a person’s quality of life.
Migraine
BACKGROUND AND OBJECTIVES: CGRP is a protein that plays a key role in causing migraines and is the target of several migraine medications.
Ataxia
When Behçet affects the brain (neuro-Behçet), the brainstem is often involved, which can cause unsteadiness and poor coordination (ataxia). This is one of the more common movement problems seen in neurological Behçet disease.
Venous thrombosis
Behçet disease has an unusual tendency to inflame veins and form clots (venous thrombosis), most often deep vein clots in the legs. In Behçet these clots tend to stick firmly to the inflamed vessel wall, so they are less likely than ordinary clots to break off and travel to the lungs.
Positive pathergy test
A positive 'pathergy' reaction, where the skin overreacts with a small bump or pustule after a needle prick, is a characteristic finding.
Nongranulomatous uveitis
Eye involvement in Behcet disease typically takes the form of a bilateral, recurring, non-granulomatous panuveitis (inflammation across all layers of the uvea), often with inflammation of the retinal blood vessels.
Panuveitis
Inflammation inside the eye (uveitis) is a major feature and, untreated, can threaten sight.
Erythema nodosum
Tender red nodules on the skin (erythema nodosum) are one of the recognised skin features of Behçet disease.
Pustule
Behçet commonly produces acne-like, pus-filled skin bumps and inflamed hair follicles (pseudofolliculitis). These papulopustular lesions are one of the skin signs used to help diagnose the disease.
Genital ulcers
Genital ulcers are a core feature of Behçet disease, often occurring alongside recurrent mouth ulcers and eye involvement.
Recurrent aphthous stomatitis
Behcet disease typically causes recurring mouth ulcers (oral aphthous ulcers).
Oral ulcer
Recurrent mouth ulcers are a core feature of Behçet disease. They are often described as one part of a triad alongside genital ulcers and eye involvement.
Intestinal involvement (gut ulcers)
Some people with Behçet develop ulcers in the digestive tract, typically where the small and large intestine meet. This intestinal involvement can cause belly pain, bleeding, and diarrhea, and sometimes develops over time in people who started with only mouth and skin symptoms.
How it is diagnosed
Behçet disease
Diagnosed using: International Criteria for Behcet's Disease.
“The International Criteria for Behçet's Disease were adopted as the reference…”
Behçet disease
Diagnosed using: pathergy test.
“Pathergy was positive in 8 of 12 (66.7%).”
Treatment and management
What the research describes, not a recommendation. Treatment decisions belong with your clinician.
This covers treatments that appear in the published research mapped here. Investigational and experimental therapies are not included, so their absence is a boundary of this map, not a sign they do not exist.
Apremilast
Apremilast is recommended for Behçet disease that does not respond to first-line treatment, alongside immunosuppressive medicines such as TNF-alpha inhibitors.
Used to help with: Behçet disease.
“These ulcers resolved along with the systemic symptoms following treatment with colchicine, apremilast, and prednisolone.”
colchicine
For the mouth, skin, and joint symptoms of Behçet disease, colchicine is recommended as the first-line treatment.
Used to help with: Behçet disease.
“For mucocutaneous and joint involvement, colchicine is recommended as the first-line treatment modality.”
TNF-alpha inhibitors
TNF-alpha inhibitors are recommended for Behçet disease that does not respond to first-line treatment, and early use of these monoclonal antibodies is encouraged when there is organ or life-threatening involvement.
Used to help with: Behçet disease.
“Early use of monoclonal antibodies against TNFα is encouraged in patients with organ or life-threatening manifestations.”
glucocorticoids
When Behçet disease affects internal organs, more aggressive treatment with glucocorticoids and immunosuppressive medicines is recommended to bring the disease under control quickly.
Used to help with: Behçet disease.
“For patients with organ involvement, more aggressive treatment with glucocorticoids and immunosuppressives is recommended for rapid induction of remission.”
azathioprine
Azathioprine is a steroid-sparing immunosuppressive medicine commonly used in neuro-Behçet disease, the form of Behçet disease that affects the brain and nervous system.
Used to help with: Behçet disease.
“Steroid-sparing immunosuppression (most commonly azathioprine…”
roflumilast
Roflumilast, a PDE4 inhibitor, may help treat oral ulcers in Behçet disease that have not responded to other medicines, and can be an alternative where apremilast is unavailable.
Used to help with: Behçet disease.
“Roflumilast, a phosphodiesterase-4 (PDE4) inhibitor, may be effective for treating refractory oral ulcers (OUs) in BD and…”
What changes how it shows up
The diagnosis is not the whole story. The factors and open questions below are described in the research mapped here as shaping whether, or how strongly, the condition shows up, or as points the field has not yet settled. They are not, on their own, its cause or its cure.
HLA-B51 association
HLA-B51 is a genetic marker that is more common in people with Behçet disease. It is found in only some patients and does not by itself cause or confirm the condition.
Described as modulating: Behçet disease.
“HLA B51 was positive in 24 of 37 (64.9%).”
IL-23R rs11209032 susceptibility variant
A variant in the IL-23R gene (rs11209032) has been linked to a higher chance of developing Behçet disease. It is one of several genetic factors thought to contribute, not a single cause.
Described as modulating: Behçet disease.
“…the IL-23R rs11209032 polymorphisms show a strong association with disease…”
How to read the evidence labels
Where this comes from
This guide is built from 15 published source(s). Every claim above links back to one of them. Click any source ID to read the original on PubMed.
Take it further
Printed, source-linked documents built from this condition's graph — ready to bring to an appointment or attach to a coverage request. Every claim carries its published source, the same as this guide.