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Health condition · Clinically reviewed

Behçet disease, a variable-vessel vasculitis with a long clinical shadow.

Recurrent ulcers, eye inflammation and, in some, vascular or neurological disease. Modern, stratified therapy changes the trajectory - especially in specialist centres.

A radiographer guides a patient onto the bed of an advanced 3 Tesla MRI scanner in a London imaging suite

Why trust this guide

  • 01

    Clinically reviewed

    Written by our editorial team and reviewed by a registered UK clinician before publication.

  • 02

    Sourced from guidance

    Checked against EULAR, BSR and specialist Behçet centre standards, all listed at the end.

  • 03

    Current for 2026

    Reflects modern management including TNF inhibitors, apremilast and emerging biologics for Behçet disease.

Key facts

Behçet disease at a glance.

The essentials, in plain English - what it is, who gets it, and how UK specialists frame diagnosis and care.

  • What it is

    A variable-vessel systemic vasculitis affecting arteries and veins of any size, driven by immune dysregulation.

  • Who gets it

    Highest prevalence along the historic Silk Road, with Turkey reporting the greatest global rate, though all ethnicities are affected.

  • Age and sex

    Typical onset in the 20s to 40s, with roughly equal numbers of women and men in most UK cohorts.

  • Genetics

    Strong association with HLA-B*51, though the gene is neither necessary nor sufficient to cause disease.

  • Hallmark

    Recurrent oral aphthous ulcers - three or more episodes a year - are mandatory for the ISG diagnostic criteria.

  • Care setting

    Multidisciplinary care across rheumatology, ophthalmology, neurology, vascular and gastroenterology, ideally through a specialist Behçet centre.

Why this guide matters

A rare disease with high stakes - and a strong evidence base.

Behçet is uncommon in UK primary care, so recognition matters. The three points below shape everything else on this page.

  • It is a systemic vasculitis

    Not just mouth ulcers - Behçet can inflame arteries and veins of any size, driving eye, brain, vascular and gut disease.

  • Eye disease drives morbidity

    Panuveitis and retinal vasculitis are the leading cause of long-term damage - early biologic therapy has transformed visual outcomes.

  • MDT care changes outcomes

    Rheumatology, ophthalmology, neurology, vascular and gastroenterology, ideally through an NHS Centre of Excellence - the difference is measurable.

How the diagnosis is made

From recurrent ulcers to an ISG-based diagnosis.

The steps a UK rheumatologist will normally follow, in order - so you know what to expect and why each investigation matters.

  1. 01

    Assessing

    Clinical history and mucocutaneous review

    A careful record of oral and genital ulcers, skin lesions, eye symptoms and systemic features over months to years.

  2. 02

    Assessing

    ISG criteria assessment

    Recurrent oral aphthous ulceration (three or more episodes a year) plus two of: genital ulcers, eye disease, skin lesions or a positive pathergy test.

  3. 03

    Assessing

    Pathergy test

    A sterile skin prick read at 24 to 48 hours - a papule or pustule greater than 2 mm is positive and highly specific.

  4. 04

    Confirming

    Inflammatory markers and HLA-B*51

    ESR and CRP support systemic inflammation. HLA-B*51 is supportive, not diagnostic - many patients are negative.

  5. 05

    Confirming

    Exclude mimics

    Inflammatory bowel disease, reactive arthritis, herpes simplex, SLE, polyarteritis nodosa, granulomatosis with polyangiitis and syphilis all need ruling out.

  6. 06

    Confirming

    Ophthalmology assessment

    Slit-lamp and dilated fundoscopy look for anterior uveitis, posterior uveitis and retinal vasculitis - even without eye symptoms.

  7. 07

    Preparing

    Targeted imaging

    MRI brain and MR venography if neurological features. CT or MR angiography if vascular disease is suspected, particularly pulmonary artery involvement.

Typical timeline: from suspected diagnosis to a specialist plan in weeks, coordinated through an MDT.

Symptoms

What Behçet disease looks like.

The classic mucocutaneous mix, plus the ocular, vascular, neurological and gastrointestinal features that make Behçet a truly systemic disease.

  • Recurrent oral aphthous ulcers

    Painful, round, well-defined ulcers on the tongue, gums or inner cheeks - three or more attacks a year is the diagnostic threshold.

  • Genital ulcers

    Recurrent painful ulcers on the scrotum, vulva or perineum, often leaving scars - a strong pointer to Behçet disease.

  • Skin lesions

    Pseudofolliculitis, papulopustular lesions and erythema nodosum, most often on the lower legs.

  • Eye disease

    Anterior and posterior uveitis with retinal vasculitis - the leading cause of long-term morbidity if untreated.

  • Positive pathergy

    A papule or pustule appearing 24 to 48 hours after a sterile skin prick - specific for Behçet.

  • Joint involvement

    Non-erosive, non-deforming inflammatory arthritis, most often in the knees, ankles and wrists.

  • Vascular symptoms

    Deep vein thrombosis, superficial thrombophlebitis and, less commonly, arterial aneurysms including pulmonary artery aneurysms.

  • Red flag - neurological or vascular

    New headache, focal neurology, haemoptysis or sudden visual loss needs urgent specialist assessment.

Complications

The organ systems Behçet can touch.

Recognising complications early is what changes the trajectory of the disease. Some are life or sight-threatening and warrant immediate specialist input.

  • Ocular

    Panuveitis and retinal vasculitis can cause irreversible visual loss - the leading long-term morbidity of Behçet disease.

  • Neuro-Behçet

    Parenchymal disease affects the brainstem and basal ganglia; vascular disease causes dural venous sinus thrombosis and intracranial hypertension.

  • Vascular

    Deep vein thrombosis, Budd-Chiari, aortic and peripheral aneurysms - and pulmonary artery aneurysms with life-threatening haemoptysis.

  • Gastrointestinal

    Ileo-caecal ulcers mimic Crohn’s disease and can perforate - endoscopy and imaging distinguish the two.

  • Cardiac

    Pericarditis, myocarditis, endocarditis and valvular disease can complicate long-standing Behçet.

  • Musculoskeletal

    Non-erosive inflammatory arthritis, usually of the knees, ankles and wrists - typically self-limiting but recurrent.

Treatment

How Behçet disease is treated in the UK.

Care is stratified by organ. Mucocutaneous disease responds to colchicine and apremilast; severe ocular, vascular, neurological or gastrointestinal disease usually needs biologics per EULAR guidance.

  • Colchicine

    First-line for mucocutaneous disease at 1 to 2 mg daily, reducing oral and genital ulcer frequency and erythema nodosum.

  • Topical and intralesional steroid

    Steroid mouthwash, pastes and injections give fast local relief for ulcers alongside systemic therapy.

  • Apremilast

    A PDE4 inhibitor approved in the UK for oral ulcers of Behçet disease when colchicine is inadequate.

  • Azathioprine

    A cornerstone steroid-sparing agent for eye, vascular, neurological and gastrointestinal disease.

  • Ciclosporin

    Effective for sight-threatening uveitis, though avoided when neurological Behçet is present due to neurotoxicity.

  • TNF inhibitors

    Infliximab or adalimumab - EULAR first-line for severe or refractory eye, neurological, vascular or gastrointestinal disease.

  • Interferon-alpha

    An alternative for sight-threatening uveitis, particularly when TNF inhibitors are unsuitable.

  • Cyclophosphamide and IVIg

    Reserved for severe parenchymal neuro-Behçet, refractory vascular disease and life-threatening flares.

Stratified by organ

  • Mucocutaneous. Colchicine 1-2 mg, topical steroid, intralesional steroid and apremilast for refractory oral ulcers.
  • Ocular. High-dose steroid with azathioprine or ciclosporin; infliximab or adalimumab first-line for severe eye disease per EULAR; interferon-alpha as an alternative.
  • Vascular. Steroid with azathioprine, cyclophosphamide or a TNF inhibitor; anticoagulation used selectively because of arterial aneurysm bleed risk; endovascular or surgical repair for selected aneurysms.
  • Neuro. Steroid with azathioprine, cyclophosphamide or a TNF inhibitor; IVIg in selected cases.
  • Gastrointestinal. 5-ASA, steroid and azathioprine; TNF inhibitors for refractory disease; surgery for perforation or fistula.
  • Emerging. Secukinumab, ustekinumab, tocilizumab and JAK inhibitors are in trials and specialist use.

All patients benefit from MDT input across rheumatology, ophthalmology, neurology, vascular and gastroenterology, ideally through an NHS Behçet Syndrome Centre of Excellence at the Royal London, Liverpool or Birmingham.

What this guide is based on

The sources behind every claim on this page.

EULAR and BSR guidance, NHS England commissioning standards, and Behçet’s UK patient resources, current at the time of last review.

Key references

Guidelines and standards we relied on.

A quiet reminder

This guide is for information, not medical advice.

Your rheumatologist or specialist team knows your case in detail and can tell you which parts apply to you. If in doubt, get seen.

  • EULAR. Recommendations for the management of Behçet syndrome (2018 update).

  • British Society for Rheumatology (BSR). Guidance on vasculitis and Behçet disease.

  • NHS England. Highly specialised service for Behçet syndrome - Centres of Excellence.

  • Behçet’s UK. Patient information and centre network.

  • MHRA. Apremilast licence and safety information for Behçet oral ulcers.

Red flags

When Behçet needs urgent attention.

Many symptoms can be managed in outpatient settings. These are the ones that cannot - and where same-day specialist input is needed.

  • Sudden visual change

    Blurring, floaters or visual loss can signal sight-threatening posterior uveitis or retinal vasculitis - emergency ophthalmology review.

  • Haemoptysis

    Coughing blood in a patient with Behçet raises concern for pulmonary artery aneurysm - a life-threatening emergency needing urgent CT angiography.

  • New severe headache or focal neurology

    Parenchymal or vascular neuro-Behçet can present with brainstem signs, seizures or dural venous sinus thrombosis - urgent neuroimaging.

  • Painful, swollen limb

    Deep vein thrombosis is common in Behçet - anticoagulation is used selectively because of arterial aneurysm bleed risk.

  • Abdominal pain or GI bleeding

    Ileo-caecal ulcers can mimic Crohn’s and perforate - urgent gastroenterology and surgical review.

  • Chest pain or breathlessness

    Pericarditis, myocarditis or valvular disease can complicate Behçet - do not dismiss cardiac symptoms.

  • Severe oral ulceration preventing eating

    Warrants escalation to systemic therapy - apremilast or biologics rather than repeated topical courses.

  • Pregnancy planning

    Several treatments including cyclophosphamide and methotrexate are teratogenic - specialist pre-conception counselling is essential.

  • Pathergy after surgery

    Behçet patients can develop wound complications - flag the diagnosis to any surgical team in advance.

Living with it

A relapsing condition, with a clear MDT plan.

Four things that make the biggest difference year to year - accepting the rhythm of relapse and remission, working with a specialist centre, keeping ophthalmology on side, and using the patient community.

A quiet reminder

Continuity of care beats crisis care, every time.

A named consultant and a specialist centre make the difference in a disease as multi-system as this one.

  1. 01 Rhythm

    Expect a relapsing course

    Behçet flares and settles - a steady maintenance plan matters more than reacting to every ulcer.

  2. 02 Team

    Use a specialist centre

    The Royal London, Liverpool and Birmingham NHS Behçet Centres of Excellence coordinate rheumatology, ophthalmology, neurology and vascular care.

  3. 03 Vigilance

    Eye checks even when you feel fine

    Silent posterior uveitis and retinal vasculitis can damage vision without symptoms - regular ophthalmology review is non-negotiable.

  4. 04 Support

    Lean on Behçet’s UK

    Patient community, helpline and up-to-date resources from the national charity make a real difference to day-to-day life.

Frequently asked

Everything we get asked about Behçet disease.

Quick answers on diagnosis, pathergy testing, complications, treatment and NHS specialist care.

  • What is Behçet disease?

    Behçet disease is a chronic, relapsing, variable-vessel systemic vasculitis. It can affect arteries and veins of any size and typically causes recurrent oral and genital ulcers, skin lesions, eye inflammation and, in some, neurological, vascular or gastrointestinal disease.

  • Who gets Behçet disease?

    It is most common along the historic Silk Road, with the highest global prevalence in Turkey, but occurs in all ethnicities. Onset is usually between the 20s and 40s and women and men are affected roughly equally in UK cohorts. HLA-B*51 is strongly associated but not required for diagnosis.

  • How is Behçet disease diagnosed?

    Diagnosis is clinical, using the International Study Group criteria: recurrent oral aphthous ulcers (three or more episodes a year) plus at least two of recurrent genital ulcers, defined eye disease, defined skin lesions or a positive pathergy test. Inflammatory markers, HLA-B*51 and imaging support the picture and help exclude mimics.

  • What is a pathergy test?

    A sterile needle prick is made in the forearm skin and read at 24 to 48 hours. A papule or pustule greater than 2 mm at the site is a positive result. It is highly specific for Behçet disease but not universally positive - rates are higher in Turkish and Middle Eastern cohorts than in UK patients.

  • How is Behçet disease treated?

    Treatment is stratified by organ involvement. Mucocutaneous disease responds to colchicine, topical steroids and apremilast. Eye, neurological, vascular and gastrointestinal disease usually needs steroids with azathioprine, ciclosporin, interferon-alpha or a TNF inhibitor such as infliximab or adalimumab. EULAR recommends TNF inhibitors as first-line for severe eye disease.

  • Where can I get NHS specialist care in the UK?

    NHS England commissions Behçet Syndrome Centres of Excellence at the Royal London, Liverpool and Birmingham, with additional links across the country. Referral is via your GP or local rheumatology or ophthalmology team. Behçet’s UK maintains up-to-date information on services.

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