Contents
Introduction
Behçet’s Disease is a rare autoimmune disorder, causing the systemic inflammation of blood vessels. Most commonly seen in individuals of middle eastern origin, the condition is named after Turkish dermatologist Hulusi Behçet who described in 1937.
Signs, Symptoms and Pathology
- Rare in western populations, but affects about 2 per 1,000 in the Middle East
- Idiopathic, autoimmune disease, caused by inflammation of venules.
- Associated with HLA-B51, thought to be triggered by an environmental agent
- Symptoms include:
- Recurrent mouth and genital ulcers
- Uveitis
- Skin rashes and ulceration
- Genital Ulcers
- Often accompanied by a symmetrical arthritis of the lower limbs!
Diagnostic Criteria
At least 3 episode or oral ulceration 12 months, PLUS TWO of:
- Genital ulceration
- Uveitis or retinal vasculitis
- Skin lesions – either erythema nodosum (EN)-like lesions, acneiform lesions, pustulosis, or pseudofolliculitis
- Positive pathergy test (see below)
Investigations
- Bloods often normal
- May be difficult to differentiate from other autoimmune pathologies, and a autoimmune disease screen would be recommended, including: FBC, UEC, CRP, ESR, anti-nuclear antibodies, rheumatoid factor, neutrophil cytoplasmic antibodies, anti-desmoglein antibodies
- You should also consider syhpilis as a differential
- Positive Pathergy test. ‘Pathergy’ refers to exaggerated skin trauma as the result of a minor injury or bruise. This can result in serious ulceration after some types of trauma (e.g. after surgical incision). In a pathergy test, small pin pricks are made with a sterile lesion, usually in the forearm. The test is considered positive if any of the following subsequently occur:
- Red bump (papule)
- Pustule
- Ulceration
- Biopsy can be considered – usually shows dense infiltration of lymphocytes and other cells associated with inflammation within the vessel walls
- There is no specific serology for Behçet’s Disease
Management
- Non-erosive, and commonly with steroids, and other immunosuppressants
- Colchicine may also reduce the frequency of attacks