Behcet Disease

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Welcome to PT 635 Pathophysiology of Complex Patient Problems This is a wiki created by and for the students in the School of Physical Therapy at Bellarmine University in Louisville KY. Please do not edit unless you are involved in this project, but please come back in the near future to check out new information!!

Original Editors - Jennifer Edwards from Bellarmine University's Pathophysiology of Complex Patient Problems project.

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Definition/Description

A syndrome (or sometimes referred to as a disease) that causes inflammation in the blood vessels of the body. The most common symptoms are sores in the mouth, sex organs, and on the skin, as well as swelling in the eye, pain, stiffness, and swelling in the joints. The more serious complications include meningitis, blood clots, inflammation of the GI tract, and blindness. ^1,2

Prevalence

Occurs in people among the " Silk Road"- in the eastern Mediterranean, Middle East,and East Asia. Most common in females aged 25-30 years. Though when in males, it is usually a more sever form. ³

Characteristics/Clinical Presentation

The patient could present with any of the five most common symptoms of Behcet's disease.

• Mouth sores- usually the first and most persistent symptom.  They are similar to canker sores but have a longer duration and are usually larger and more painful.
• Genital sores-  less common, but still occur in more than half of the patients with this disease.  They are usually mistaken as genital herpes and can be on the external or internal surface of the genitalia.  
  
• Skin lesions- are red, raised bumps or bruises that resemble boils or acne.  They usually appear on the legs and upper torso. 
• Uvelitis and retinal vasculitis- occuring in half the Behcet's patients, this can cause blurred vision, pain, redness, or blindness if left untreated. 
• Arthritis- it results in inflammation in the joints leading to edema, heat, redness, and pain. It occurs in half the Behcet's patients and lasts a few weeks.

In addition to these five common clinical symptoms that patients present with, the circulatory, central nervous systems, and digestive tract can be affected. 

• Circulatory system- DVT, aneurysms
• Central nervous system- may experience fever, headache, stiff neck, and difficulty coordinating movements secondary to inflammation in the meninges in the brain and spinal cord. 
• Digestive tract- may lead to abdominal pain, diarrhea, and bleeding due to inflammation and ulcers in GI tract. These symptoms are similar to ulcerative colitis and Crohn's disease. ³

Associated Co-morbidities

Include: ulcerations in mouth, genitals, and GI tract, skin lesions, lung aneurysms, arthralgias, headaches, confusion, strokes, personality changes, dementia, and aseptic meningitis, anterior/posterior uveitis ⁴

Medications

For the disease that's confined to the mucocutaneous areas (mouth, genitals, skin) use topical steroids and non immunosuppressive medications. 

Moderate dosage of corticosteroids are used for exacerbations.

For some patients with chronic pain, low doses of prednisone are indicated. 

Serious organ involvement calls for high doses of Prednisone and immunosuppressive treatment.

Immunosuppressive medications include: azathrioprine, cyclosporine, cyclophosphamide, and chlorambucil ⁴

Diagnostic Tests/Lab Tests/Lab Values

Diagnosis is very difficult and may takes months or even years to get an accurate diagnosis. Key symptoms are:

• Mouth sores at least three times in one year.
• Any two of the following:
•      Recurring genital sores
•      Eye inflammation with loss of vision
•      Characteristic skin lesions
•      Positive pathergy test

A dermatologist may do a biopsy of the mouth, genitals, and skin lesions. ³

 

Etiology/Causes

The etiology is unknown but thought to be secondary to an autoimmune reaction, where the immune system attacks and harms the body's tissues, resulting in inflammation. Some thoughts are that it could have a genetic component making some more susceptible to a possible environmental trigger such as a virus or bacterium.  ³

Systemic Involvement

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Medical Management (current best evidence)

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Physical Therapy Management (current best evidence)

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Alternative/Holistic Management (current best evidence)

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Differential Diagnosis

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Case Reports/ Case Studies

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Resources

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Recent Related Research (from Pubmed)

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References

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Behcet's disease. National Institute of Arthritis and Musculoskeletal and Skin Diseases. 2009. http://www.niams.nih.gov

Behcet's syndrome. Medline Plus. 2011. Available at http://www.nlm.nih.gov. Accessed February 22, 2011.

Behcet's disease: how is it diagnosed? Arthritis Foundation. 2011. Available at http://www.arthritis.org. Accessed February 22, 2011.

Hellmann D. Behcet's disease. The John Hopkins Vasculitis Center. 2001. www.hopkinsvasculitis.org.