In 1937, Prof. Dr. It was defined by Hulusi Behçet as a disease characterized by recurrent aphthous ulcer, genital ulceration and uveitis. Its prevalence varies in different parts of the world. It has a distinct geographic distribution following the historical silk road. In different studies conducted in Turkey, the prevalence varied between 10000/8-42. In a study conducted with 2313 patients at Ankara University Faculty of Medicine Behçet Center, oral aphthae and erythema nodosum were frequently seen in women; Vascular, ocular, neurological involvement, thrombophlebitis and papulopustular reaction were found frequently in men. The disease usually appears in the 3rd or 4th decade. The disease may progress aggressively, especially in young male patients; Therefore, this age group needs to be treated more intensively.
Diagnosis : It is put clinically. In 1990, the International Behçet’s disease working group defined the diagnostic criteria. Recurrent aphthous stomatitis: Presence of two of the following symptoms in addition to the oral ulcer observed by the physician or the patient and recurring at least 3 times a year Recurrent genital ulcer {C}{C}3. {C}Skin lesions (Erythema nodosum, papulopustular eruption, superficial thrombophlebitis)
Eye findings:Anterior uveitis, posterior uveitis, vitreous cells in eye examination, retinal vasculitis Pathergy test positive in 48 hours
pathogenesis:
It is accepted that Behçet’s disease is a vasculitis triggered by immunological mechanisms. Its pathogenesis is not fully elucidated. The view that has come to the fore in recent years is that both innate and acquired immune responses play a role in the pathogenesis. The most common link with the disease has been shown with the HLA-B51 gene. However, the role of HLAB51 in the pathogenesis of the disease is unclear.
Skin findings:
Aphthous lesions : The most common symptom is recurrent aphthous ulcers. They are usually localized in the back of the mouth such as lips, cheek mucosa, gums, tongue, uvula orophainx. They can be of minor, major and herpetiform type. In the minor form, aphthous lesions are smaller than 1 cm, covered with a yellow membrane, and erythematous around it. It usually heals within 1 week without leaving any scars. Major aphthous lesions vary between 1-3 cm in diameter, take longer to heal, and may leave scarring.
Genital ulceration: 85% of people diagnosed with Behçet’s disease have genital ulcers. It has similar clinical features with major aphthae located in the oral mucosa, heals by leaving scarring. There may be more than one. In men, it can be localized in the scrotum, penis, inguinal region. In women, it can be localized in the labium major, minor, and more rarely in the vagina. It may cause adhesions in the vaginal introitus due to the scarring that occurs while healing.
Papulopustular lesions: It is mainly localized on the face, back, chest, shoulder, femoral region. It is difficult to distinguish from acne vulgaris. There is intense neutrophil infiltration around the hair follicle.
Erythema nodosum: It is seen in approximately 50% of patients. It is mostly located on the legs and arms. They are erythematous nodules that are painful and tender to touch. Sometimes it can heal by ulcerating and leaving pigmentation. Histopathologically, there is a dense infiltration of neutrophils in the subcutaneous tissue.
Superficial thrombophlebitis: It can involve large or small veins. Thrombus can be felt inside the vein with palpation. The vena safena manga is the most frequently involved vein.
Pathergy reaction: It is the appearance of a papule or pustule with an erythematous halo around 24-48 hours after inserting a 20 gauge sterile injection needle into the forearm or glutea to reach the dermis. Erythema alone is not significant. It is more severe in male patients. The rate of positivity increases with the use of blunt needles. Other findings in the disease include arthritis, ocular involvement, gastrointestinal system involvement, neurological involvement, vascular system involvement in which aneurysm and thrombosis can be seen.
Eye involvement: Uveitis can be seen in 60-80% of patients. Blindness is one of the most important complications of Behcet’s disease.
neurological involvement : It can be seen in 10-30% of patients. The prognosis is very poor. Meningitis can be seen as meningoencephalitis. Psychiatric symptoms, personality change, hemiparesis, may be seen.
Vascular involvement : Both arterial and venous systems can be involved. : thrombosis or aneurysm may develop. Its incidence is between 7-40%. Venous involvement can often be seen in the lower extremity as superficial thrombophlebitis or deep vein thrombosis. Vena cava thrombosis can lead to Budd-Chiari syndrome.
Gastrointestinal system involvement: It may present with pyoderma gangrenosum and papulopustular lesions. Ulceration, ischemia and infarction may develop due to vascular involvement in the ileocecal region. There is a risk of perforation.
Treatment:
Treatment varies according to the clinical findings of the patient. Cytotoxic drugs are preferred in patients with ocular, central nervous system and vascular involvement. The aim is to reduce mortality and morbidity.
colchicine: Oral and genital ulceration, other skin findings, eye involvement
Corticosteroid (can be applied as topical, oral, parenteral and intraocular injection),
Low dose:2nd option in the treatment of erythema nodosum, anterior uveitis and arthritis
High dose: First-line therapy for gastrointestinal involvement, acute meningoencephalitis, chronic progressive central nervous system involvement, and arthritis. Azathioprine, cyclophosphamide, cyclosporine, TNF alpha inhibitors are other drugs that can be used in the treatment of retinal vasculitis and venous thrombosis.
