Behcet's disease is a multifaceted syndrome that is characterized by ulcerations in the mouth, genitals, and uveitis. The disease frequently occurs in areas with latitudes between 30 and 45 degrees north, especially around Mediterranean basin. Behcet's disease is believed to have been spread by merchants who were travelling along ancient Silk Road and adjacent trading routes.This research paper provides detailed information about Behcet's disease. Some of the issues discussed in this research paper include the causes of the disease, prevalence of Behcet's disease, and the role of environmental factors in the likelihood of developing a disease. The signs and symptoms of Behcet's disease, such as sores in the mouth and on the genitals, swelling or inflammation of parts of the eyes and arthritis, are also discussed. Various medications that are used for treating Behcet's disease are also discussed in the paper. Moreover, the effects and complications that may result from Behcet's disease and current research studies conducted on the disease are also reviewed.
Introduction
Behcet's disease refers to a systematic vasculitis that is characterized by sporadic ulcerations of the mouth, skin, and sex organs. Ulcerations may also impact the joints, central nervous system (CNS), and gastrointestinal tract. Cade describes Behcet's disease as a rare, chronic inflammatory disorder, the cause of which is unknown (134). Behcet's disease is sometimes referred to as Behcet's syndrome, Morbus Behcet, or Silk Road disease. According to Lee, Behcet's disease was named after a Turkish dermatologist Hulusi Behcet in 1937 because he was the first person to diagnose and describe the disease (37). Hulusi Behcet recognized Behcet's disease in one of his patients in early 1924. Hulusi Behcet described the disease as complex recurrent ulcerations in the mouth and genitals that can lead to blindness. After recognizing this unique syndrome, Hulusi Behcet conducted further research studies for more than ten years before reporting his findings in the Journal of Skin and Venereal Diseases in 1936 (Lee 44). According to Yazici & Hassan, the name Morbus Behcet was adopted in September 1947 during the International Congress of Dermatology in Geneva (156).
Behcet's disease is systematic and may affect visceral organs such as the gastrointestinal tract, pulmonary, cardiovascular, musculoskeletal, and neurological systems (Yazici, Hassan 159). Behcet's disease can be fatal because it may cause massive rupturing of vascular aneurysms. Death may also result from severe neurological complications as a result of a disease. Behcet's disease is mostly common in parts of the Mediterranean, Turkey, and Japan. Zouboulis also agrees that Behcet's disease is more common in countries in the Middle East, especially in Turkey, and in countries such as Japan, North Korea, and South Korea in the Far East region (81). However, the disease is relatively rare in the United States of America and has never been reported in Africa. According to Zouboulis, Behcet's disease is more prevalent along the Silk Road, which is an ancient trading route between the Mediterranean and East Asia (83). Behcet's disease is more prevalent among Turkish and Japanese people, and it is least reported among individuals of African origin. Although Behcet's disease may affect all people equally, Yazici and Hassan argue that the disease is more common among youths aged between 18 and 27 years and middle-aged adults in their thirties (161). Zouboulis also asserts that Behcet's disease is more common among adults and least common among children (87). Behcet's disease is also relatively rare among elderly people aged above 55 years. According to Zouboulis, Behcet's disease usually begins at the age of 20 years, although the disease can occur at any age (90). Behcet's disease tends to occur more often in men than in women.
Causes of Behcet's Disease
According to the National Institute of Arthritis and Musculoskeletal and Skin Diseases, the main cause of Behcet's disease is unknown (55). It is presumed that the disease is caused by the inflammation of blood vessels in the body. However, medical scientists and physicians have not established what causes inflammation of the blood vessels. It is also presumed that people with impaired immune systems are likely to develop Behcet's disease when they come into contact with substances such as bacteria and viruses. Behcet's disease is incommunicable and cannot be spread from one person to another. Behcet's disease has also been associated with exposure to toxins. To date, no particular virus has been isolated to cause Behcet's disease.
Prevalence of Behcet's Disease
In Turkey, the prevalence rate is estimated to be between 200 and 500 individuals per one hundred thousand individuals, whereas in Japan, the prevalence of Behcet's disease is estimated to be between 60 and 100 per 100 000 individuals. In Mediterranean, Behcet's disease is more common among men, while in Asia, Behcet's disease mostly affects women.
Behcet's disease also affects people who have positive family history of the disease. This suggests that people who have a family member suffering from Behcet's disease are more likely to get infected by it.
Role of Environmental Factors on Infection by Behcet's Disease
Although the main cause of Behcet's disease is unknown, recent research studies suggest that both genetic and environmental factors play an important role in infection by the disease. For instance, a study which was conducted among migrants between 1997 and 2002 revealed that the surrounding of an individual has a reasonable impact on susceptibility of Behcet's disease. In the study, six hundred individuals who migrated from Turkey to Germany showed reduced vulnerability to the disease. Moreover, the disease is rare in the United States of America and has never been reported in Africa. Thus, geographical location affects the rate of infection by the disease.
Signs and Symptoms of Behcet's Disease
The most common signs and symptoms of Behcet's disease are ulcerations of the mouth and genitals (Zierhut, Ohno 120). Painful oral mucocutaneous ulcerations usually occur in the form of aphthous ulcers. Hoffman also affirms that early stages of development of Behcet's disease are also characterized by oral ulcerations (215). According to Zierhut and Ohno, oral ulcers in Behcet's disease are similar to mouth ulcers in appearance and localizations. However, oral ulcerations may be more painful and extensive than mouth ulcers (127). Oral ulcers usually evolve very quickly from flat, small ulcers to large sores. Oral ulcers mostly affect the tongue, lips, and gingiva. Oral ulcers in Behcet's disease may also affect buccal mucosa, pharynx, and tonsil (see Figure I below).
A patient of Behcet's disease would also develop painful genital ulcerations around the anus, vulva, and scrotum. These genital ulcerations usually cause scarring in more than 75 percent of patients with Behcet's disease. A patient may also develop inflammatory eye disease (see Figure II below) during the early stages of development of Behcet's disease. Approximately 30 to 70 percent of patients with Behcet's disease suffer from inflammation of eyes. According to Hoffman, ocular involvement is more common and severe among male patients than among female patients (217) (see Chart I below). The National Institute of Arthritis and Musculoskeletal and Skin Diseases also stress that inflammatory eye disease may lead to permanent loss of vision (61). Inflammation of eyes, which is one of the most common symptoms of Behcet's disease occurs in more than 70 percent of all reported cases. This makes it a major criterion upon which the diagnosis of Behcet's disease is based.
It is also estimated that approximately 80 percent of patients with Behcet's disease develop skin diseases. In addition, nearly two-thirds of patients of Behcet's disease suffer from articular diseases. The most common joint manifestation associated with Behcet's disease includes synovitis, arthritis, and arthralgia. Most articular diseases caused by Behcet's disease are non-deforming and often affect knees, ankles, and wrists. Female patients usually suffer from erythem a nodosum, which often affects lower limbs.
Behcet's disease also causes vascular diseases that affect both arteries and veins. The pathological process of Behcet's disease is characterized by small-vessel vasculitis. On the other hand, less than 40 percent of patients with Behcet's disease suffer from large vessel vasculitis. Diseases affecting the veins, such as superficial thrombophlebitis and deep venous thrombosis, are also common among patients with Behcet's disease.
Approximately 10 percent of patients of Behcet's disease develop infections of central nervous system (CNS). According to the National Institute of Arthritis and Musculoskeletal and Skin Diseases, neurological manifestations usually occur within 5 years of onset of Behcet's disease (66). According to Hoffman, infections involving gastrointestinal tract often vary among patients of Behcet's disease. Infections of the gastrointestinal tract are more common among Japanese patients than Turkish patients (220).
Most of the signs and symptoms of Behcet's disease subside spontaneously or without treatment only to reoccur later. As Behcet's disease progresses, a patient is likely to develop more serious symptoms, which often vary from one person to another. For example, severe cases of infection by Behcet's disease may be characterized by inflammation of the brain and spinal cord, presence of blood clots in the body, inflammation of the digestive systems, which lead to indigestion and loss of vision as a result of inflammation of eyes. In addition to the above-described infections of various organs by Behcet's disease, many patients also experience fatigue and malaise. Some patients also experience massive weight and recurrent fever. The signs and symptoms of Behcet's disease may also appear and disappear from time to time. Behcet's disease is also characterized by alternating periods of activeness and inactiveness.
Diagnosis of Behcet's Disease
There is no single diagnostic test for Behcet's disease that is currently known. According to the National Institute of Arthritis and Musculoskeletal and Skin Diseases, several laboratory tests that have been studied did not show any single diagnostic test for the disease (70). Thus, the diagnosis of Behcet's disease is usually based on the findings of careful clinical examinations of the patient. A review of history of family and health of the patient is also important. According to Zierhut, Ohno, the diagnosis of Behcet's disease is extremely difficult and challenging because of three major reasons (134). Firstly, the symptoms do not usually appear all at once. Secondly, there are other illnesses such as inflammatory bowel disease and pyoderma gangrenosum, which exhibit similar symptoms thus causing confusion during diagnosis. Thirdly, there is no single test that has been invented for diagnosing Behcet's disease.
It is widely held that Behcet's disease commonly affect people with impaired immune systems, who are genetically susceptible to the disease. Behcet's disease has also been consistently associated with polymorphisms in the human leukocyte antigen complex.
Prevention and Treatment of Behcet's Disease
There is no known method of preventing the development of Behcet's disease. According to the National Institute of Arthritis and Musculoskeletal and Skin Diseases, many favored treatments for Behcet's disease still lack a sound evidential base (72). However, cyclosporine and azathioprine are the most preferred medications that have proved useful during clinical trials for the treatment of Behcet's disease. Medical research has also been gathering evidence to indicate that anti-tumor necrosis factor therapy can also be used as an alternative treatment for Behcet's disease.The National Institute of Arthritis and Musculoskeletal and Skin Diseases also suggests that colchicines, non-steroidal anti-inflammatory drugs, corticosteroids, and immuno-suppressants can also be used to treat Behcet's disease (74-75).
Specialized therapy can also be used in some cases. Inflammation of the eyes can be treated using corticosteroid eye drops. However, the National Institute of Arthritis and Musculoskeletal and Skin Diseases warns that corticosteroids alone are not sufficient for prolonged treatment or for the prevention of blindness, which results from Behcet's disease (78). He further claims that corticosteroids are often used together with cyclosporine. Chlorambucil in combination with corticosteroids has also been proved to be effective in treating ocular lesions. Corticosteroids are usually administered to help reduce pain and inflammation, while immunosuppressive drugs are administered to help control infections of the immune system, reduce inflammation of the immune system, and prevent disease flares.
In addition, Petrov puts forward that Behcet's disease affects many parts of the body (371). Therefore, patients are advised to visit different doctors in order to receive specialized treatment. The most common doctors who treat patients from Behcet's disease include dermatologists who treat skin disorders, ophthalmologists, who deal with eye infections, gastroenterologists, who deal with infections of gastrointestinal or digestive tract, rheumatologists, who treat arthritis and other inflammatory disorders, and gynecologists, who deal with infections of sexual organs of women. Patients are also advised to seek treatment from neurologists, hematologists, and urologists. According to Zierhut, Ohno, Behcet's disease is incurable (139), thus the goal of treatment is to reduce pain and prevent serious complications. Patients with Behcet's disease are also often encouraged to be actively involved in moderate physical exercises, such as swimming and walking, to facilitate quick recovery.
Effects and Complications of Behcet's Disease
The most common effects of Behcet's disease include inflammation of the skin, blindness, stroke, and swelling of the spinal cord. Complications of gastrointestinal tract have also been associated with the disease. Behcet's disease may also cause various types of skin lesions, arthritis, painful joints, and inflammation of bowels. Meningitis, which refers to inflammation of the membranes of the brain and spinal cord, is also common among patients with Behcet's disease. According to Petrov, Behcet's is a multi-system disease, which may affect several organs vital systems, particularly the central nervous system of the body (385).
Although symptoms of Behcet's disease are usually mild, the disease may become chronic in cases where vital body organs, such as the central nervous system ,which includes the brain and the spinal cord and large blood vessels, are involved. Lee also asserts that involvement of gastrointestinal tract and central nervous systems during Behcet's disease is often life-threatening (164). Behcet's disease may also lead to memory loss and impaired speech because of infection of the central nervous system.
Further Research on Behcet's Disease
There are numerous research studies conducted on Behcet's disease. In the recent past, medical researchers and scientists have been investigating how the drugs currently used for treating Behcet's disease would be improved and the role of certain genes in the development of Behcet's disease. Moreover, researchers have been exploring the role of environmental factors such as geographical location and exposure to certain bacteria and virus in the cause and development of Behcet's disease.
Conclusion
Although Behcet's disease is a rare disease whose diagnosis is relatively difficult and challenging, doctors should strive to ensure that the disease is accurately diagnosed at the early stages of development and properly treated to prevent more serious complications, which may lead to loss of lives.I would also suggest that treatment for Behcet's disease should be symptomatic and supportive. Medications that are prescribed by doctors should aim at reducing ulcerations in the body as well as help boost the immune system of the patient. Other treatment methods such as immunosuppressive therapy may also be considered.
Last but not least, I would urge that further research studies should also be conducted to find out ways of reducing or eliminating complications of Behcet's disease, for example, new drugs should be invented to help eliminate more serious complications of the disease such as meningitis, blood clots, inflammation of the digestive system, and blindness.
