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Behçet's Disease

Behçet's disease is characterized by systemic vasculitis and is the result of autoimmunity. It is a rare type of disease that involves the ulceration of the mucous membranes that line the eyes and genitalia. Other names for this disease include Morbus Behçet, Behçet's Syndrome or Silk Road disease.

Prevalence and Course

Males are more likely to develop this disease. It is estimated that each year 10,000 - 15,000 Americans are diagnosed with this disease. In the UK, it is estimated that 1 in 100,000 people are affected by this condition. Behçet's disease occurs more frequently in individuals between the age of 20 and 30 but can affect people of any age. Individuals with this disease usually develop eye disease at the age of 30.

Behçet's Disease takes long to progress since it is an autoimmune mediated disease; this makes it not easy to cure and as a result an individual usually lives with the condition for life (the condition often comes and goes but never completely vanishes).


Description

It was named after Hulusi Behçet, a Turkish dermatologist in the year 1937. Hulusi Behçet first explained the triad of the symptom complex characterized by the recurrence of attacks of genital ulcers, oral aphthous ulcers and uveitis. Since it is a systemic disease, it also involves different visceral organs e.g. the gut, respiratory tract, neurological and musculoskeletal systems. Systemic diseases include arthritis, meningitis, bowel inflammation, cranial nerve palsies and different types of lesions developing on the skin. This disease can prove fatal if it leads to a rupturing of vascular aneurysms, or different types neurological complications.


Symptoms

Patients with Hulusi Behçet are presented with painful oral ulcers also called aphthous ulcers. In addition to oral ulcers, there are painful genital ulcerations present around the vulva, scrotum or anus. These genital ulcerations lead to scarring in a large number of patients. A rash on the skin can also be seen in patients together with eye inflammation. There may also be swelling and pain of the joints.  


Causes of Hulusi Behçet

The exact cause of Behçet's Disease is not yet know and is thought to be the result of an autoimmune condition. Autoimmunity affects the blood vessels and produces inflammation within the vessels which further leads to the development of different painful symptoms. Genetic’s seem to play a part in the development of this disease as it runs in the first degree of relatives of the affected patient.


When to See a Doctor

If you develop a lesion on the oral mucous (membranes of the mouth) and it does not heel and at the same time you feel the formation of another lesion on your genitalia or eyes, you should seek the help of a health care provider.


Diagnosis

Since there is no diagnostic tests available for this disease, the following criteria can be used to diagnose Behçet's Disease:

  • Oral ulcers developing at least three times a year
  • The presence of any 2 of the following pathologies
    • Genital ulcers of recurrent variety
    • Inflammation of the eyes leading to blindness
    • Distinctive skin lesions
    • A positive skin prick test


Treatment of Hulusi Behçet

Treatment is usually symptomatic since there is no curative treatment present for the Behçet's Disease. High doses of corticosteroid therapy is indicated when the disease is severe. Anti-TNF therapy such as infliximab is beneficial as it treats uveitis successfully associated with this disease.  Etanercept is another anti-TNF that provides relief in cases of skin and mucosal lesions. For genital and mouth sores interferon alfa-2a is an effective treatment.

 
 

 
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