Mr TF is a 34-year-old Turkish man. He has been experiencing very painful mouth ulcers for the past year.
He says that he has had at least eight ulcers and each one has lasted around a fortnight.
Mr TF is usually very fit and well and does not take regular medication. However, he saw one of your partners two months ago complaining of a sore right eye. He was diagnosed then as having uveitis.
He also tells you that he has had a small ulcer on his scrotum, which was also extremely painful a few months ago.
He has noticed that he has been more tired than usual recently and complains of intermittent muscle and joint pains.
You examine him and find that he has several small aphthous ulcers on his buccal mucosa. He is mildly tender around his knee and ankle joints, but you could not demonstrate any swelling or effusion of these joints.
You refer Mr TF to a local rheumatologist for further investigations.
He is subsequently diagnosed as having Behcet's disease.
Behcet's disease is a complex multisystem disorder characteristically presenting with recurrent oral ulcers. It can also involve the mucocutaneous, ocular, cardiovascular, renal, GI, pulmonary, vascular, musculoskeletal, urological and central nervous systems.
Behcet's disease is thought to be an autoimmune condition. Its prevalence is highest in the Middle East, the Mediterranean and eastern Asia. It is most common among people aged 20-30 years and affects males and females equally. However, males generally have a more active and severe form of the disease.1
It is associated with HLA-B51 and there is a genetic predisposition.
The presentation of Behcet's disease varies enormously between patients because it can affect so many different systems in the body.
Non-specific symptoms include tiredness, malaise, muscle pains and transient fevers. Oral and genital ulceration, and eye disease are the classic triad of manifestations.
The aphthous ulcers can occur on any part of the moist mucosal surfaces inside the mouth, but not on the lips. The genital ulcers are very painful and do not usually occur on the penis.
Other skin manifestations include erythema nodosum and acne-like lesions on the arms and legs.
Eye involvement includes uveitis, which occurs in around half of all cases. Patients can also develop retinal haemorrhage, retinal vasculitis, hypopyon and photophobia.
Memory impairment is the most common neurological manifestation. Neurological involvement usually occurs late in the course of this disease. Impairment of balance, speech and movement can develop. Thrombosis within the dural venous sinuses may also occur.
Some patients have a vasculitis affecting their large and/or small vessels associated with the disease.
This can lead to arterial occlusions and even aneurysms developing.
Arthralgias and a non-erosive arthritis can be common. Large and small joints can be affected. Other manifestations include diarrhoea and vomiting, epididymitis, glomerulonephritis, myositis and rarely, amyloidosis. Most new manifestations occur within the first five years after onset of the disease.
The International Study Group criteria are currently used to define the illness.2 For the diagnosis of Behcet's disease to be made, the patient needs to have recurrent oral ulcers and two of the following manifestations in the absence of other systemic diseases:
- Recurrent genital aphthous ulceration or scarring observed by a physician or reported reliably by the patient.
- Skin lesions (including erythema nodosum like lesions, pseudofolliculitis, papulopustular lesions, or acneiform nodules) observed by a physician in a post-adolescent patient not receiving corticosteroids.
- Eye involvement with anterior/posterior uveitis or retinitis observed by an ophthalmologist.
A pathergy test is sometimes also done, in which minor skin trauma induces an inflammatory papule or pustule after 24-48 hours. However, this is only positive in up to 60% of patients.
The aim of treatment is to prevent long-term damage. The most severe manifestation present usually determines the choice of treatment.
Topical corticosteroids (such as triamcinolone paste) are effective for milder oral or genital ulcerations.
Patients with more severe symptoms usually need systemic treatment, such as azathioprine, cyclophosphamide, or a TNF-alpha inhibitor (for example, infliximab).3 Disease activity diminishes with time and most patients go into remission.
- Dr Newson is a GP in the West Midlands.
1. Al-Araji A, Kidd DP. Neuro-Behcet's disease: epidemiology, clinical characteristics, and management. Lancet Neurol 2009; 8(2): 192-204.
2. Criteria for diagnosis of Behcet's disease. International Study Group for Behcet's Disease. Lancet 1990; 335(8697): 1078.
3. Fresko I, Yazici H. Treatment strategies for Behcet's disease. Expert Opin Pharmacother 2008; 9(18): 3211-19.