What is Behcets Syndrome

Some of the following texts have been translated into German by provisu. No guarantee can be given for the correctness and completeness of the original language texts and the translations have no claim to completeness, but merely serve as an information offer to the best of our knowledge and belief. Under no circumstances can they replace competent advice from a specialist.


Behçet syndrome is a rare chronic rheumatic disease that was first described by the Turkish doctor Dr Hulusi Behçet. He defined the syndrome with the following three symptoms: aphthous ulcers in the mouth, aphthous ulcers in the genital area and uveitis (inflammation in the anterior chamber of the eye). About 60-70% of patients have bilateral uveitis. Sometimes choroiditis, vitreous hemorrhage, optic neuritis, diseases of the retinal vessels and other symptoms also occur which, if left untreated, can lead to blindness. The causes of Behçet's syndrome are unknown.

source : HONselect

Adamantiades-Behçet's disease, named after a Greek ophthalmologist and a Turkish dermatologist, is a chronic, progressive inflammation of all small and large vessels. The disease occurs more frequently in people along the former Silk Road, i.e. in the eastern Mediterranean, Iran, Iraq, Saudi Arabia and the Far East.

source : www.aerztezeitung.de


Main features of the disease are mouth ulcers in almost all patients. They can grow over an inch and heal poorly. There are also acne-like skin changes, but in atypical areas. Mechanical irritation of the skin causes papulopustules in up to 15 percent of patients (pathergy phenomenon). Several erythema nodosum foci and sometimes vasculitic ulcerations can often be seen on the lower extremities. Pain in the knee joints and ankles is common.

source : www.aerztezeitung.de


In the German population, the prevalence is estimated at 0.6 to 1.5 per 100,000 inhabitants. The causes of the disease are unclear. There is a strong genetic component. The HLA antigens B51 and B27 are often found in Behçet patients.

source : www.aerztezeitung.de