Behcet's Syndrome

Turkish dermatologist, Hulusi Behcet (1889-1948) recognized and reported in 1937 symptoms of Behcet's syndrome. A dental infection is attributed as the etiology of the disease. The American Behcet's Disease Foundation (ABDA) was founded in 1978 with the objective to provide support to patients and family as well the caregivers. In the absence of a cure or a single test to definitely determine Behcet's syndrome or Behcet's disease, educate about the syndrome to seek prompt medical attention for treatment.

Morbus Behcet or Silk Road disease is the other name for Behcet Syndrome or disease. Month of May is Behcet's Awareness month and May 20th is Behcet's awareness day. The focus is on spreading awareness and stress the importance of self-help.

Behcet's syndrome facts

• Non-contagious
  
• Exact cause is unknown
  
• Genetic predisposition is high.
  
• Symptoms are similar to other disease of the digestive tract, Ulcerative Colitis and Crohn's Disease
  
• No single test to confirm diagnosis.
  
• No cure available.
  
• Disease affects different parts of the body.
  
• Men and women are equally prone but more severe in men.
  
• In the US, affects more women.
  
• More prevalent in Middle East, Mediterranean and Eastern Asia.
  
• Turkey has the highest prevalence.
  
• Leading cause of blindness in Japan.
  
• Most common among age group 20-30.
  
• Treatment requires more than one specialist.
  
• Continued, extensive research to explore possible genetic, bacterial and viral cause.
  
• Research is on to identify medicines to better treat Behcet's disease.

Behcet's syndrome – Autoimmune disease

There are over 14, 000 auto-immune diseases and 7000 plus are rare. Behcet's syndrome is rare. Autoimmune disease occurs when the body's immune system mistakenly attacks its own healthy tissues. Though the clinical feature of auto immunity is absent, Behcet's syndrome is classified as an autoimmune disease as it has various aspects related to autoimmune disease. Enhanced inflammatory response (inflammation of blood vessels) is one such aspect. Significant number of women as compared to men are more likely to be affected by autoimmune disease. Estrogen predisposes women to autoimmune disease.

Behcet's syndrome symptoms

The inflammation of blood vessels, particularly veins causes symptoms in many parts of the body. Swelling, redness, heat and pain are select features of an inflammation. Though Behcet's disease can affect any part of the body, involvement of the neurological system is known as neuro-Behcet's disease and is rated the most disabling complication of the disease. Not that common, neuro-Behcet's disease affects about 10 per cent of people with Behcet's disease.

The most common symptom of Behcet's syndrome is the regular occurrence of ulcers in the mouth and genitals. The symptoms are an off-shoot of inflammation of the eyes, skin, arteries, veins, joints, nervous and digestive systems and heart. Symptoms can be mild or severe. Symptoms can be noticed between ages 20-30 years.

Mouth ulcers: May look like normal mouth ulcers but are more painful and numerous in number. Ulcers develop in the tongue, lips, and gums and inside of the cheeks. Even if the ulcers heal within a couple of weeks, they recur.

Genital ulcers: In men, though genital ulcers can appear anywhere in the groin area, including the penis, it is more common on the scrotum. In women, the ulcers appear on the cervix (neck of the womb), vulva or vagina. The ulcers are usually painful and scars appear around the area.

Skin lesions: Resembling acne, pustular skin lesions can appear anywhere on the body. Erythema nodosum results in red, painful, tender lumps that can measure one to five centimeters. It is the result of inflammation in the fatty layer of the skin. It can appear on the legs and ankles but can also appear on the face, neck or arms. Erythema nodosum related to other disease heal without scars but if related to Behcet's disease, leaves the skin totally discolored.

Inflammation of the eyes: Sudden inflammation of the eyes is a common symptom of Behcet's syndrome. A group of connected tissues, uveal tract is inside the eye. This uveal tract gets inflamed. Uveitis as it is named can cause symptoms such as, painful red eyes, blurred vision and floaters (dots that move across the field of vision). With a possibility of permanent visual impairment, it is best to seek medical attention for appropriate treatment without any delay.

Skin sensitivity: Pathergy is a condition signifying sensitivity of the skin, particularly injury or irritation. Even a needle prick can lead to developing a large red lump in a day or two.

Gastrointestinal symptoms: Inflammation of the stomach and intestines causes symptoms such as loss of appetite, stomach pain, indigestion, diarrhea, feeling sick and vomiting. There is a possibility of damage to the bowel resulting in bleeding. Blood in stools suggests inflammation of the internal lining of the bowel.

Blood clots: Inflammation of the veins can lead to formation of blood clots or thrombosis.

Joint pain: Joint pain in ankles, wrists, knees, elbows and hips is common. Inflammation in the joint can cause swelling, redness and tenderness.

Brain: Meninges is the coverage of the brain. Inflammation of the brain or tissue that covers the brain (meninges) causes symptoms like headache, neck stiffness along with high body temperature. In severe cases, it can damage the nervous tissue and lead to extreme weakness or impaired function of the body.

Aneurysms: Aneurysms are outpouchings of blood vessel walls due to inflammation of arteries in the lungs. This can lead to massive lung hemorrhage. Symptoms include pain in the limbs, severe headache, feeling dizzy, and breathlessness, coughing up blood, confusion and loss of consciousness.

Inflammation of the nervous system: Inflammation of the central nervous system occurs in 5%-10% of reported cases. This is regarded as the most serious symptom. Typical symptoms include headache, double vision, loss of balance, seizures, partial paralysis on one side of the body, personality changes. Any of these are noticeable within the first five years of recognizing initial symptoms.

General symptoms: Experiencing extreme fatigue to the extent that it interrupts with everyday routine is a general symptom of Behcet's syndrome.

Behcet's Syndrome Causes

The exact cause remains unclear. Though regarded as an autoimmune disorder, it is unclear what triggers the autoimmune disorder. Other possible association is genetics. Certain ethnic groups or a family member with the disease increases chances of developing Behcet's syndrome. People with gene HLA B51, variations in other genes like IL10, IL23R-IL2RB2 increases the risk of developing the disease considerably. However, the condition does not have a clear pattern of incidence.

Risk factors identified include:

Age: Children and older adults can develop the condition. But men and women in the age group of 20-3 are more likely to be affected.

Location: People from countries in the Middle East and Far East, including Turkey, Iran, Japan and China are more likely to develop Behcet's syndrome.

Sex: The disease is more severe in men.

Genes: Having certain genes increases the risk of developing the syndrome.

Behcet's Syndrome Diagnosis

In the absence of a single test to diagnose Behcet's syndrome, doctors look out for symptoms. Blood tests or other laboratory tests are recommended to rule out other disease or illness. The criteria include:

Mouth sores: Mouth sores are very common. Many disorders are related to mouth sores. If the mouth sores recur every three months in 12 months, it is regarded as criteria for evaluating the disease.

Doctors look for two additional signs.

• Genital Sores
  
• Eye problems
  
• Skin sores
  
• Pathergy test

Behcet's Syndrome - time to seek help

Recognizing symptoms and relating the symptoms to Behcet's disease is essential. Note down symptoms being experienced and add related information that affects normal routine. Seek appointment with a doctor. If medical attention is delayed, the condition can worsen and lead to losing eye sight or a stroke. At the meeting, divulge family history and inform about medications for any other health issue or supplements being taken.

You are most likely to seek specialist help. Rheumatologist for arthritis, joint pain etc, Ophthalmologist for eye problems, gynecologist or an urologist for genital sores, dermatologist for skin issues, Gastroenterologist for digestive difficulties or neurologist for symptoms related to CNS (central nervous system).

Behcet's Syndrome Treatment

There is no cure for Behcet's syndrome. The treatment is aimed at alleviating the symptoms, reducing the frequency and intensity, put the disease into remission and prevent serious complications. Medicines for controlling the individual symptoms are prescribed. Some medicines are prescribed with other medicines to suppress the activity of immune system. Some medicines can have side effects too.

For the skin: gels, creams and ointment that contain a Corticosteroid to reduce the inflammation.

For mouth sores: Special mouth washes with Corticosteroid to reduce the pain and associated discomfort.

For the eyes: Eye drops with Corticosteroid to relieve pain and redness in the eye.

Coping with Behcet's syndrome

• Take ample rest in between flares.
  
• Pay attention to diet and exercise.
  
• Find support groups and connect.
  
• Login to American Behcet's Disease Association.
  
• Read message boards and engage in chat sessions.

Pathergy

Pathergy refers to an abnormal reaction of the skin that occurs in response to minimal trauma. In Pathergy, integrity of tissue is severely disrupted at the site of injury, resulting in exaggerated inflammatory condition. The response to the trauma in Pathergy phenomena is very severe and prominent compared to the normal skin.

Pathergy can also occur at the site of surgical incisions and cause ulcerations. Pathergy condition is more prevalent along the Silk Route, which spans from Japan and China in the Far East to the Mediterranean Sea, including countries such as Turkey and Iran.

Pathergy is often associated with Behcet Disease and also to an extent with Pyoderma Gangrenosum and Sweet Syndrome. The presence of Pathergy reaction is usually diagnosed with the help of Pathergy test (PT). In a Pathergy test, a sterile needle is inserted into the skin and the site is left for observation for one or two days. Skin of the forearm is normally chosen to perform the test. If the pathergy test is positive, a small red bump or pustule forms under the skin where the needle was inserted. This indicates dysfunction of the immune system and its overreaction to a minor injury. A blunt needle is recommended over a sharp one for better diagnosis of Pathergy.

Positive Pathergy is an important criteria for diagnosis of Behcet Disease. However, the test by itself does not confirm Behcet Disease. It is only indicative and has to be supported by other diagnostic procedures.

Erythema Nodosum

Inflammation of the skin that shows up as painful reddish tender lumps is called Erythema nodosum. This inflammation is usually located in a part of the fatty layer of skin (subcutaneous fat). The size of the lump could vary in size from 1 to 5 cm. The inflammation causes nodular swelling. The inflammation remains for about a week and then becomes flat leaving behind a bruised appearance. They usually show up on the shins (front portion of the legs, just below the knee). Erythema nodosum is a type of panniculitis, i.e. inflammation that can cause nodules below the surface of the skin. The condition is more common among youngsters aged between 12-20 years.

Erythema nodosum settles down on its own after a period of three to six weeks. It may leave behind a temporary bruised appearance or a chronic indentation in the part where the fatty layer has been injured. Though the condition is annoying and painful, the condition does not cause any damage to the internal organs of our body. In adults, the condition is more often seen in women than in men. In kids, it affects boys and girls equally. In a few people the trigger can be identified and in yet a few it cannot be identified. However identifying the trigger becomes very important as it needs to be treated.

Erythema nodosum causes

Erythema nodosum may show up on its own or may occur in association with other conditions. In about 30-50% of cases, the cause is unknown. However the common triggers that may cause Erythema nodosum include:

• Step throat (streptococcal infection) in adults and kids
  
• Fungal diseases
  
• Tuberculosis
  
• Cat scratch disease
  
• Few medications like penicillin, oral contraceptive, Hepatitis B vaccination, etc
  
• Sarcoidosis
  
• Inflammatory bowel diseases
  
• Behcet's disease
  
• Pregnancy
  
• Certain types of cancers like lymphoma, leukemia, etc

Erythema nodosum symptoms

• Painful nodules are commonly seen on the shin and sometimes on the arms, thighs and trunk of the body. The nodule when it first appears is red, hot and firm to touch and becomes flat over a few days. It appears as bruise that is blue in color and later turns yellowish as it fades. A person may develop just a couple of nodules or much more.
  
• Flu-like feeling before the appearance of lumps.
  
• Aching joints and legs before the appearance of lumps. Ankle, knee and wrist joints are the most affected.
  
• Loss of weight.
  
• Symptoms may vary depending on the underlying trigger that caused the condition.

Erythema nodosum diagnosis

• By means of physical examination
  
• Biopsy of the affected area
  
• Blood test to identify the trigger
  
• Chest X-ray in case of TB or sarcoidosis induced condition
  
• Throat swab

Erythema nodosum treatment

• Cortisone injection
  
• Oral cortisone
  
• Anti-inflammatory drugs
  
• Bed rest
  
• Compression bandages
  
• Cool wet compresses