Behcet's Disease

Question 1

What is Behcet’s disease?

Answer 1

Behçet’s disease is a complex inflammatory condition.

It characteristically presents with recurrent oral and genital ulcers.

It can also cause inflammation in a number of other areas such as the skin, gastrointestinal tract, lungs, blood vessels, musculoskeletal system and central nervous system.

The presentation can vary a lot between patients, with some patients mildly affected and others affected dramatically.

Question 2

What gene is Behct’s associated with?

Answer 2

HLA B51

This as prognostic indicator of severe disease

Question 3

Differentials of Behcet’s

Answer 3

Simple aphthous ulcers are very common

Inflammatory bowel disease (particularly Crohn’s disease)

Coeliac disease

Vitamin deficiency (B12, folate or iron)

Herpes simplex ulcers

Hand, foot and mouth disease (coxsackie A virus)

Squamous cell carcinoma

Question 4

Common clinical features of Behcet’s

Answer 4

Mouth Ulcers
Patients with Behçet’s disease are expected to get at least 3 episodes of oral ulcers per year.
They are painful, sharply circumscribed erosions with a red halo. They occur on the oral mucosa and heals over 2-4 weeks.

Genital Ulcers
Genital ulcers are similar in appearance to the oral ulcers. “Kissing ulcers” are where an ulcer develops on two opposing surfaces so that they are facing each other.

Question 5

Other clinical features.

Answer 5

Skin
Erythema nodosum
Papules and pustules (similar to acne)
Vasculitic type rashes

Eyes
Need emergency review by ophthalmology as they can be sight treating.
Anterior or posterior uveitis
Retinal vasculitis
Retinal haemorrhage

MSK
Morning stiffness
Arthralgia
Oligoarthritis often affecting the knee or ankle. This causes swelling without joint destruction.

GI
Inflammation and ulceration can occur through the gastrointestinal tract.

CNS
Memory impairment
Headaches and migraines
Aseptic meningitis
Meningoencephalitis

Veins
Can become inflamed and this can lead to vein thrombosis. Examples of this are:
Budd Chiari syndrome
Deep vein thrombosis
Thrombus in pulmonary veins
Cerebral venous sinus thrombosis

Pulmonary artery aneurysms can develop. If they rupture this can be fatal.

Question 6

Ix of Behcet’s

Answer 6

Behçet’s disease is a clinical diagnosis based on the features of the condition.
The only particular investigation to be aware of is the pathergy test.

The pathergy test involves using a sterile needle to create a subcutaneous abrasion on the forearm. This is then reviewed 24 – 48 hours later to look for a weal 5mm or more in size.

It tests for non-specific hypersensitive in the skin. It is positive in Behçet’s disease, Sweet’s syndrome and pyoderma gangrenosum.

Question 7

Management of Behcet’s

Answer 7

Referral to whatever symptoms are present.

Topical steroids to mouth ulcers (e.g. soluble betamethasone tablets)

Systemic steroids (i.e. oral prednisolone)

Colchicine is usually effective as an anti-inflammatory to treat symptoms

Topical anaesthetics for genital ulcers (e.g. lidocaine ointment)

Immunosuppressants such as azathioprine

Biologic therapy such as infliximab

Question 8

Prognosis

Answer 8

Behçet’s disease is a relapsing remitting condition.
Patients generally have a normal life expectancy and the condition may go in to complete remission.
There is an increased mortality with haemoptysis, neurological involvement and other major complications.