Behcet's disease is an acute autoimmune and mucocutaneous disease. Read the article to know about the causes, clinical signs and symptoms, investigations, systemic and orogenital manifestations, and its management by multispeciality approach.
Behcets disease or Behcets syndrome or silk road disease is rare but of a worldwide prevalence, especially along the silk road countries. It mainly affects the younger and middle-aged population between 20 to 40 years. It is a chronic syndrome and an inflammatory disorder involving the ocular, oral, and genital regions of the body. It is more prevalent between 20 to 40 years of age and with increased prevalence in men than in women. The other name for this syndrome is malignant apthosis. This mucocutaneous inflammatory disorder with uveitis and systemic vasculitis affects arteries and veins, leading to a multisystemic manifestation of a broadened disease spectrum.
The increased prevalence of this disease across the silk route gave it the name of the silk road disease (in people originating from the ancient trading routes between the china and Mediterranean basin). Though attributable genetic elements have been identified like HLA-B1 or B5 carriers who are at an increased predisposition to develop this disease, other factors like tumor necrosis factor or TNF, histocompatibility class 1 chain genes, and heat shock proteins have also been found to be carriers that may be inherited over a familial route. This autoimmune inflammatory condition is known to be due to the pathogenesis of the body's natural immune cells mistaking the healthy tissue cells to be foreign and attacking the blood vessels leading to systemic vasculitis.
Cell-mediated immunity in which the Type 1 helper T cell activation leads to increased circulating levels of T-lymphocytes accounts for the multisystemic presentation of Behcet disease. Pro-inflammatory cytokines (IL-1, IL-8, IL-12, IL-17, IL-37, and TNF) are involved in the pathophysiology behind the increased inflammatory response.
Mucocutaneous lesions, including oral aphthous ulcers and erythema nodosum, are attributed or researched due to this increased neutrophil activation (leading to a neutrophilic vascular reaction that causes tissue injury to the body's healthy tissue cells). Circulating immune complexes play a role in causing neutrophil stimulated vasculitis. Biopsy of the mucocutaneous lesions shows a predominance of neutrophil cells apart from endothelial swelling seen histopathologically.
Erythrocytes or the red blood cell extravasation is also a pathologic finding, along with fibrinoid necrosis of the blood vessels involved. The neutrophilic vascular reaction is the diagnostic criterion histopathologically in Behcet disease. The formation of aneurysms in the large arteries or vasculitis is a well-observed clinical feature. Exposure of certain infectious microorganisms like Streptococcus bacterium, Staphylococcus aureus, and Prevotella sp has remained elusive in linkage to Behcet's disease. However, research suggests that they may have an environmental role in causing systemic infection.
The main clinical symptoms that should prompt the patient to consult the physician in this condition are usually the occurrence of these features:
Reddening of the eyes
Joint stiffness or joint swellings.
Diagnosis of Behcet's disease can be made by-
Complete blood count.
Routine blood examination.
CT (computed tomography) or MRI (magnetic resonance imaging) scan.
Pathergy test (in which the skin tissue is needle pricked to observe and check if red spots begin to appear within 24 to 48 hours of the test time).
Bilateral uveitis of the anterior and posterior uveal tracts.
Vision loss may be observed in progressive disease cases (untreated).
Retinal vasculitis - leading to vision loss or blindness.
Conjunctivitis (maybe rare but observed as well).
Severe retinal disease. ·
Oligoarthritis or polyarthritis or monoarthritis.
Peripheral joint and spine involvement (arthritis).
Vascular and cardiac manifestations:
Superficial or deep thrombophlebitis.
Coronary artery vasculitis.
Budd-Chiari syndrome or vena cava obstruction.
Vasculitis of carotid, femoral and the popliteal arteries.
Elevated protein in the cerebrospinal fluid.
Cerebellar and pyramidal tract involvement.
Cranial or peripheral nerve involvement.
Headaches and papilledema.
Mucosal ulcerations of ileum, cecum, colon, and esophagus.
Ileocecal ulcerations or lesions.
Other systemic manifestations:
Inflammatory marker elevation.
These are the most commonly reported and observed clinical features in a patient presenting with Behcet syndrome. Oral lesions are not only painful but may occur anywhere in the regions of the soft palate, hard palate, buccal mucosa, lips, gingival tissue, or the tonsils. These lesions are both painful and recurrent, and routine dental examination can also prompt the dental surgeon to suspect severe systemic illnesses associated with an inflammatory immune response presenting in the oral cavity. Oral ulcerations are not only indicative or confirmative in almost all patients presenting from Behcet disease but also are the first clinical features to present in the patient.
Genital ulcerations occur in up to 80% of affected patients and are recurrent. However, in sharp contrast to the nature of oral lesions or ulcers, genital ulcers are usually self-healing with a bit of scarring. These ulcers occur in the scrotum region of the males and the females upon the vulva or the vaginal parts.
Systemic lupus erythematosus ( SLE).
Inflammatory bowel disease (IBD).
Axial inflammatory arthritis.
Behcet's disease is always associated with a significant physical and psychological impairment for life. Hence, multisystemic management should be provided which depends on medical intervention by the physician, gastroenterologist, dentist or maxillofacial surgeon, nephrologist, cardiologist, and neurologist as per the extent of organ damage and clinical symptoms exhibited by the individual. Younger individuals, especially the male population affected, need immediate treatment after detection of Behcet's disease. Otherwise, the long-term prognosis will be affected due to the high mortality rates. Hence early intervention is required. The treatment varies widely on the organ affected, ranging from corticosteroids to immunosuppressive agents. Surgery is mainly used to treat pulmonary or neural aneurysms (localized swelling of arteries are called aneurysms) and prevent their recurrence.
To conclude, Behcet's disease is a lifelong systemic disease that needs multi-specialty intervention, psychological support, and, most importantly, diagnosis at the earliest to prevent the high rates of mortality risk that arise due to the complications developed by these individuals.
No, Behcet’s disease is not sexually transmitted. It is a rare inflammatory disorder that is more prevalent among men. However, its clinical manifestation might mimic a sexually transmitted disease. The classic sign and symptoms include:
- Painful ulcers in the mouth.
- Skin rashes.
- Genital ulcer.
- Pain and swelling in joints.
Behcet’s disease is rare, and no specific treatment completely cures it. However, most affected individuals survive their whole lives with the symptoms present. The particular symptoms of Behcet’s keep recurring. Rarely in 5 % of cases, the disease becomes serious, causing severe systemic complications. The condition turns life-threatening if bowel perforation and blood vessel rupture occur.
Behcet’s disease is an anti-inflammatory disease characterized by the inflammation of blood vessels in the body. It results in various signs and symptoms in men and women. Of which the sores in the mouth are the most common sign. The mouth sore may be single or multiple and can vary in size. The mouth sore appears round and raised with reddish borders. It turns into a painful ulcer and causes discomfort while eating and swallowing. These lesions may heal within weeks but may leave scars. The mouth sores keep recurring.
Behcet’s disease causes several symptoms that occur in remissions. Various studies show that the following components may trigger it:
- Genetic factors.
- Environmental factors.
- Immune response.
Researchers have found that stress is one of the most triggering factors that are mainly responsible for causing mouth sores in Behcet's disease.
Behcet’s disease may not be diagnosed using a specific laboratory or radiographic investigation. But these tests may aid in differentiating Behcet’s disease from other autoimmune disorders. The healthcare specialist may ask you about the characteristics of symptoms, as the oral and genital ulcers due to Behcet’s disease may occur two to three times a year. The following symptoms are noticed while diagnosing Behcet’s disease:
- Genital ulcer.
- Skin rash.
- Eye inflammation.
The oral ulcer as a result of Behcet’s disease may recur due to the following foods:
Behcet’s disease causes inflammation of blood vessels in all regions of the body. Therefore, it results in various signs and symptoms that occur in remissions. Many affected individuals complain of extreme tiredness, swelling, and pain in the joints. Most commonly, the knee joint is affected. Other areas include the elbows, ankles, and wrist. These symptoms may last for three weeks and later resolves independently.
Behcet’s disease is not treatable with specific medications; however, the doctor suggests a few during the flare-up of the disease to control its symptoms. It includes:
- Topical corticosteroids to manage skin problems and genital sores.
- Systemic corticosteroids to reduce inflammation in joints.
- Mouthwashes and rinses to reduce the discomfort due to oral sores.
- Eye drops containing corticosteroids to reduce redness and inflammation in the eyes.
The primary clinical manifestation of Behcet’s disease is that it produces arthritis-like symptoms. It causes swelling, redness, and pain mainly in the knee, wrist, and elbow joints. The joint pain and swelling may last for weeks and subsides independently. These symptoms may occur due to an abnormal immune system that attacks the body’s healthy cells and tissues. Several factors like diet, stress, and genetic changes may trigger the recurrence of symptoms. The doctor may suggest corticosteroids and other physical therapy to relieve such joint pain.
Behcet’s disease is also known to affect the eyes, and it includes the following:
- Blurry vision.
- Mild sensitivity to light.
- In severe cases, the vision is greatly affected.
Several studies have reported that the symptoms of Behcet’s disease, such as oral ulcers and joint pain, are triggered by the following components:
Foods like nuts, pineapple, peanuts, and cheese.
Therefore, the doctor may suggest you stay away from such triggering factors to reduce the flare-up of the disease.
Behcet’s disease is rare, and due to inflammation of blood vessels, it causes eye, joint, skin, oral, and genital problems. The symptoms are chronic, and treatment focuses on reducing their severity. However, very rarely, it may lead to the following complications:
- Decreased or loss of vision.
- Vascular problems.
- Weakened blood vessel.
Last reviewed at:
30 Jun 2022 - 5 min read
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