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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.



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.

Gingivitis

Gingivitis involves inflammation of the gums surrounding the teeth. Due to this, the gums become soft and swollen and usually red instead of a healthy pink. The inflammation of the gums is a defense mechanism put up by the body's immune system to prevent the growth of bacteria which along with the remnant food particles and plaque form tartar. This cannot be removed by simple brushing or flossing.


What Causes Gingivitis Disease?

The primary cause of gum disease is plaque. However, there are various other reasons why gingivitis disease develops.


  • Gums become sensitive when there are hormonal changes in the body, such as during puberty, pregnancy, menopause and menstruation. During pregnancy, hormonal changes and tartar induce excessive growth of gum tissues resulting in the formation of lumps commonly referred to as pregnancy tumors. These tumors prevent bacteria from being removed effectively during brushing, thus resulting in gingivitis disease.

  • In post-menopausal women, a painful condition called desquamative gingivitis may develop, for reasons yet unknown. In this condition, the outer layers of the gums come away from the teeth and lose their solidity, resulting in the exposure of nerve-endings which causes acute pain.

  • Long-term use of contraceptive pills can also result in gingivitis.

  • Diseases that affect the immune system such as HIV or cancer may also affect the gums. Diabetes when uncontrolled, can affect the gums. On the other hand, gingivitis disease may also be a pointer to systemic disorders.

  • Some medications can also cause gum disease as they restrict production of saliva which prevent the formation of tartar. Some medications used in the treatment of angina and seizures can cause abnormal growth of the tissues in the gums.

  • Habits, such as smoking, do not facilitate the easy self-repair of the gums. A severe form of gingivitis, known as Trench mouth or ANUG (Acute necrotizing ulcerative gingivitis) is mostly found in the case of smokers. ANUG is also reported in the case of incapacitated persons under severe stress. Acute pain, swelling, bleeding and bad breath develop very quickly in this condition and also the gums turn grey.

  • If gingivitis runs in the family, then there is a greater chance of a person getting it. Poor oral hygiene is a major contributor to gingivitis.

  • Dental appliances not properly fitted or improper alignment of teeth may also cause gingivitis.

  • Dietary imbalances, vitamin deficiencies, and drug reactions can also result in gingivitis.

  • Gingivitis disease is also often caused by fungal and viral infections.


Symptoms of gingivitis

There is usually no pain associated with mild form of gingivitis. Hence it may go unnoticed. However, there are certain symptoms that warn that treatment for gingivitis is needed:


  • When the gums are swollen, soft, or red.

  • When gums bleed during, or after brushing.

  • When bad breath or bad taste in the mouth persists even after brushing and flossing.

  • When the gums recede.

  • Deep pockets are formed between teeth and gums.

  • Visible deposits of tartar.



If the dentist finds that someone is particularly prone to formation of plaque, he may recommend use of special implements to remove plaque, such as toothpicks, electric toothbrushes etc. This treatment for gingivitis would suffice when the gingivitis disease is in the early stages.

For reducing the pocket depth between teeth and gums, which can also cause gingivitis, dentists undertake root-planing and scaling. In addition dentists may also administer minocycline microspheres. For women who have desquamatic gingivitis, hormone replacement therapy could be beneficial. However, adverse effects of the therapy could prevent their recommendation. Instead, corticosteroid rinses and pastes which can be directly applied on gums may be more commonly recommended.

Pericoronitis is acute, painful inflammation of the gingival tissue over a partly erupted tooth (as in the case of a wisdom tooth). The treatment for this would include removal of the debris beneath the gingival flap and irrigation with saline. This may be followed up with extraction, particularly when there is recurrence of the episode.


Leukoderma

Leukoderma or vitiligo as it is sometimes called, is a chronic skin condition that causes loss of pigment leading to pale spots of skin. Leukoderma can be described as an autoimmune skin disease where the body destroys its own pigment cells, melanocytes. After the pigment disappears, small or even larger areas of skin become white with sharp margins where they join unaffected parts of skin. The hair in these areas also grow white rather than pigmented. Leukoderma is more noticeable during summer when the normal skin darkens. The causes for leukoderma could be a combination of genetic and environmental factors. Inheritance seems to be a dominant trait for this condition.


Leukoderma may also occur at sites of injury to the skin and may show itself as scars or burns. Recent studies suggest that leukoderma could be caused by the development of an antibody to an enzyme in the pigment cell. Leukoderma may be caused due to several other factors as well - congenital as in tuberous sclerosis, partial albinism and Piebaldism and Waardenburg's syndrome. The immunological causes of leukoderma are Vitiligo and Halo mole. Thermal burns, Dermatitis or eczema and Psoriasis may also result in leukoderma. Infectious conditions such as Pityriasis versicolor, leprosy, lichen planus and syphilis could also result in leukoderma. Some occupational hazards that could cause leukoderma are exposure to depigmentation agents such as tertiary butyl phenol and exposure to chemicals.


The spots can spread, shrink or remain the same. It is often noticed that patches occur in symmetrical fashion across both sides of the body. Some times mild trauma to an area of skin can cause new spots as around ankles caused by friction due to shoes or sneakers. Corticosteroid ointment or cream of appropriate strength is used depending on the site involved. Normally a mild steroid is used on the face and a stronger one for the trunk and limbs. A specialized form of light treatment PUVA is also of value to some patients.

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Collection of Pages - Last revised Date: November 17, 2019