What is Behçet’s disease?

Behçet’s disease 


It is also called Behçet’s syndrome. It is a vasculitis of unknown cause which targets small arteries and venules. It is characterized by recurrent mouth and genital ulcers, ocular lesions, and skin, joint & neurological involvement. It is a recurrent, multifocal disorder that persists over many years.


It is rare in Western Europe but more common in ‘Silk Route’ countries, around the Mediterranean and in Japan. It has a strong association with HLA-B51. Association with B12, DR2, DR7 and Drw52 are also found. 


Clinical features of Behçet’s disease 


  • Oral and genital ulcers: These are present in most of the patients. Oral ulcers are universal; deep and multiple, and last for 10–30 days. Ulcers can affect the pharynx and cause dysphagia. Genital ulcers are found in 60–80% of the patients and can cause dysuria and dyspareunia.
  • Eye involvement: Ocular lesions are the most serious development. Recurrent uveitis ( anterior and posterior both) and iridocyclitis, retinal vascular lesions and optic atrophy can lead to loss of vision in 50% of patients with ocular involvement
  • Skin lesions: The usual skin lesions are erythema nodosum. Other skin manifestations include diffuse pustular rash affecting the face (acneiform lesions), erythema multiforme, features of migratory thrombophlebitis and vasculitis etc
  • Joints involvement: Seronegative arthritis (mono or oligo-arthritis) affects about 40% of patients and commonly involves knees, ankles, wrists and elbows 
  • Vascular involvement: Recurrent thrombophlebitis of the legs is a significant feature of Behçet’s syndrome, leading to venous thrombosis. Less often, superior or inferior caval thrombosis may occur
  • GIT involvement: Anorexia, abdominal pain and bloody diarrhoea have also been documented
  • Neurological involvement: Neurological complications occur in 20% of patients. Organic confusional states, meningoencephalitis, transient or persistent brainstem syndromes, multiple sclerosis and parkinsonian-type disorders are all recognized

Diagnosis of Behçet’s disease: 


  • It is a clinical diagnosis. There is no specific diagnostic test except for the pathergy test.
  • Pathergy test: It is specific to Behçet’s disease. Pricking the skin with a needle leads to the development of pustular lesion within 24–48 hours.
  • ESR and CRP are usually elevated
  • Asymptomatic proteinuria is found

Treatment of Behçet’s disease


  • Oral ulceration can be managed with topical glucocorticoid preparations (soluble prednisolone mouthwashes, glucocorticoid pastes)
  • Colchicine can be effective for erythema nodosum and arthralgia
  • Thalidomide (100–300 mg per day for 28 days initially) is very effective for resistant oral and genital ulceration but is teratogenic and neurotoxic
  • Glucocorticoids and immunosuppressants are indicated for uveitis and neurological
  • Anti-TNF agents can be used to control severe uveitis and serious manifestations such as neurological and gastrointestinal Behçet's disease.

Diagnostic criteria for Behçet’s disease (in the image): 



Diagnostic criteria for Behcet's disease





Source:

  • Davidson's Principles and Practice of Medicine; 23rd Edition; page 1043
  • Kumar and Clark's clinical medicine, 9th Edition; page 702
  • Rapid review of clinical medicine for MRCP part 2, 2nd Edition, by Sanjay Sharma and Rashmi Kaushal; Q 31

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