How does the disease manifest itself?
In almost all patients, the disease begins with aphthae - round ulcers with a whitish bottom and a red rim on the mucous membrane of the cheeks, lips, and tongue. They heal in one to three weeks and come back again. Ulcers on the genitals are less common, but leave scars. Painful red nodes on the legs, acne-like and pustular rashes appear on the skin. The joints become inflamed without cartilage destruction. The most serious manifestations are uveitis, venous thrombosis, pulmonary aneurysms and damage to the nervous system.
- Aphthous ulcers in the mouth are a mandatory sign
- Ulcers on the genitals
- Erythema nodosum and pustular rash
- Uveitis and other eye inflammations
- Arthritis of large joints without destruction
- Venous thrombosis, less commonly arterial aneurysm
- Headaches and neurological disorders
Causes and risk factors
The exact reason is unknown. The disease is thought to be the result of an excessive immune response in a genetically predisposed individual, probably triggered by oral microbial factors. Carriers of a certain variant of the HLA-B51 gene are noticeably more likely to get sick, but many healthy people have it, so the analysis itself does not confirm the diagnosis. The disease is not contagious and is not transmitted by contact. In men, it is usually more severe, with more frequent damage to the eyes and blood vessels.
- Genetic predisposition, HLA-B51 variant
- Onset usually occurs between 20 and 40 years of age
- More severe course in young men
- Oral microbial factors as a possible trigger
- Geographical distribution along the Silk Road
Eye damage is the main threat
Inflammation of the choroid occurs in a significant proportion of patients and can occur almost without pain, gradually reducing vision. Posterior uveitis involving the retina and its vessels is especially dangerous: without treatment, it leads to permanent loss of vision. Therefore, an examination by an ophthalmologist with a dilated pupil is indicated when making a diagnosis and regularly thereafter, even if there are no complaints. Any sudden clouding, appearance of floaters, pain or redness of the eye is a reason to contact us on the same day.
- Anterior uveitis: pain, photophobia, redness
- Posterior uveitis and retinal vasculitis are often painless
- Floating flies and veil - an early signal
- Routine fundus examination at least as prescribed by a doctor
- Timely treatment preserves vision
Diagnostics
There is no test that confirms Behçet's disease. The diagnosis is clinical: it is made by the combination of recurrent oral aphthae with genital ulcers, eye lesions, skin rashes, or a positive pathergy test. The pathergy test is an injection with a sterile needle into the skin of the forearm, after which a papule or pustule forms within a day or two. Blood tests show only nonspecific inflammation and are needed to assess activity and exclude other causes. Additionally, the doctor excludes infections, inflammatory bowel diseases and other vasculitis.
- Assessing the recurrence and nature of ulcers
- Examination by an ophthalmologist with fundus
- Complete blood count, ESR, C-reactive protein
- Pathergy test
- HLA-B51 typing as an auxiliary method
- Ultrasound of veins and visualization of vessels for suspected thrombosis
Treatment and self-help
The amount of therapy depends on which organs are involved. For isolated ulcers and skin manifestations, topical glucocorticoids and colchicine are used. In case of damage to the eyes, blood vessels, intestines or nervous system, systemic glucocorticoids are prescribed along with immunosuppressants, and if the effect is insufficient, genetically engineered biological drugs are prescribed. The treatment is selected by the doctor, it is long-term, and it cannot be stopped if it improves. The patient is helped by careful oral hygiene, treatment of caries, avoidance of foods that injure the mucous membrane and smoking.
- Topical glucocorticoids for ulcers
- Colchicine for mucocutaneous and joint manifestations
- Systemic glucocorticoids for organ damage
- Immunosuppressants and genetically engineered drugs
- Thorough oral hygiene and dental sanitation
- Quitting smoking and spicy foods
- Routine examinations by an ophthalmologist and rheumatologist