Behcet’s disease is a type of chronic vasculitis (inflammation of blood vessels) that manifests itself through mouth, skin, eyes, and genital ulcers. It develops through acute and recurrent outbreaks of inflammation. Behçet’s disease is neither hereditary nor contagious. Its incidence varies greatly from country to country, with Turkey and Japan being the most affected countries. It is much rarer in the US.
Behcet’s disease Courtesy of Ahmet Altiner MD, Rajni Mandal MD
Behcet’s disease: symptoms
Behcet’s disease is usually defined by a typical triad of symptoms:
Mouth and genital sores
Mouth sores that look like canker sores are very common and recurrent. The typical lesion is round (between 1 and 3 cm in diameter), with a well-defined erythematous margin and covered with a yellowish-white film. In the mouth, mouth ulcers can affect the tongue, gums, lip, and oral mucosa. In the genital area, lesions appear on the scrotum and penis in men and on the vulva in women. They are often very painful and can leave scars.
Skin ulcerations
Erythema nodosum (painful subcutaneous nodules), pseudofolliculitis (pustules not centered by a hair follicle), acne-like pimples, superficial Migratory Thrombophlebitis. The skin becomes hyperreactive to all aggressions (injections, scratches, allergy tests).
Eye lesions
This is the most serious symptom of Behcet’s disease. The inflammation of the eye affects about 60% of patients with a blindness rate of 10 to 15%. It manifests itself as uveitis, intraocular suppuration, photophobia, tears, or red eyes. These repeated inflammations can lead to changes in the iris and retina, which can result in a reduction or even loss of vision.
Other symptoms may also occur:
Joints
Arthralgia (Pain in Joints), which mainly affects the large joints (knees, wrists, ankles, elbows). They are recurrent and in most cases asymmetrical.
Digestive issues
Anorexia, nausea and vomiting, diarrhea, abdominal pain. The symptoms are quite similar to Crohn’s disease.
Venous damage
Venous thrombosis most commonly in the lower limbs, stomach, and head. Arterial thromboses are less frequent (3 to 5% of cases), with a high risk of aneurysm.
Neurological deficiency
This is the main cause of death associated with Behcet’s disease. It affects 20% of patients within one to ten years after the first signs of the disease. It is then called ” neuro-Behcet’s disease “. It takes different forms: meningitis or meningoencephalitis, cerebral nerve palsy, cerebral venous thrombosis. These disorders are responsible for headaches, loss of consciousness, confusion (disorientation), paralysis of the facial muscles (disorders of eye movement and swallowing…), epilepsy, personality changes, and dementia.
Behcet’s disease: causes and origin
The causes of Behcet’s disease are still unknown today. The most common hypothesis is that it is an inflammation that is triggered by a viral or bacterial infection in people that are genetically disposed to Behcet’s disease. The disease is characterized by an infiltration of lymphocytes and neutrophils into the affected organs, which may be activated by an infectious pathogen. It is also known that this pathological infiltration depends on a genetic predisposition. The HLA B51 antigen is thus found in 50% to 70% of patients.
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Behçet’s disease: Diagnosis and treatment
There is no specific test for Behcet’s disease. The diagnosis is based on the presence of the classic triad mentioned above and the elimination of other possible causes of inflammation. Treatment consists mainly of alleviating the symptoms. It depends very much on the clinical manifestations and must be individualized. For example, colchicine is used to reduce the frequency of mouth and genital ulcers. In severe forms, anti-inflammatory corticosteroid drugs are prescribed during eruptions. Recent treatments include anti-TNF-alpha, an antibody-based treatment that inhibits the action of TNF-alpha, a protein involved in inflammation, and immune responses.
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It is very important to go see a doctor if you have any of the symptoms of Behcet’s disease as, without treatment, the eye damage can become irreversible and can lead to blindness, arterial ruptures may be fatal and the neurological disorders suffered may lead to a loss of autonomy.
References
https://www.mayoclinic.org/diseases-conditions/behcets-disease/symptoms-causes/syc-20351326
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4584512/
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