Behçet's disease

The Behçet's Disease (often Adamantiades - Behçet ) is a relapsing immune mediated disease of the rheumatic diseases. Heaped Turkish and Southeast Asian men are affected, the disease peaks is beyond the third decade of life. But even young people can become ill with them of Behcet's disease runs a long monosymptomatic. The incidence is < 1/100.000. The cause of the disease is not fully understood. It is suspected that a genetic predisposition in combination with exogenous factors ( viral or bacterial infection) leads to disruption of immune regulation and thus to the onset of the disease.

Historical Aspects

Even in ancient times was by Hippocrates mentions a disease that can be identified with the Adamantiades - Behçet probably. The first modern description comes from H. Planner and F. Remenovsky ( Archives of Dermatology and Syphilis, Berlin 1922). A summary description of William Gilbert ( German ophthalmologist, born 1879) was published in 1925 ( Archives of Ophthalmology, Wiesbaden 1925). Named the disease after Benedictos Adamantiades ( Greek ophthalmologist, 1875-1962 ) and is Hulusi Behçet (Turkish dermatologist, 1889-1948 ). Adamantiades published 1931-1936 several descriptions (among Annales d' oculistique, Paris 1931). Behçet was the first to propose to regard the symptom complex as a separate entity ( Dermatological weekly, Hamburg 1937). Another acclaimed description comes from Albert Touraine (French dermatologist, 1883-1961; La presse médicale, Paris 1955).

Pathophysiology

The disease is probably based on an autoimmune -mediated vasculitis. There are mainly affected capillaries and veins, especially in the vessels of the mucous membranes of the eyes and skin are affected. Involvement of the gastrointestinal tract is possible as well as the occurrence of arthritis and myositis. In the later course of the disease may lead to involvement of intracranial structures in the form of an infestation of the brain substance or supplying the brain vessels.

Symptoms

Oral and genital aphthous ulcers, which recur at regular intervals, may be first signs of illness. Other symptoms are collections of pus in the anterior chamber ( hypopyon ) and rainbow skin inflammation ( iritis ), which can lead to blindness after several years. Other ophthalmic manifestations are conjunctivitis, keratitis, uveitis, and optic neuritis. Other rheumatic symptoms such as localized skin redness ( erythema ) and nodules in the skin (erythema nodosum ), joint pain and inflammation, thrombophlebitis, venous and arterial occlusions, aphthous lesions in the lower small intestine, or epididymal inflammation ( epididymitis ) are also described in connection with Behçet's Disease been. When meningoencephalitis ( parenchymatous form) occurrence of the brain stem and basal ganglia are preferentially affected. It comes to headache, incoordination, spastic paresis and impaired consciousness. As a result intracranial vascular form sinus or cerebral venous thrombosis.

Diagnosis

Even after minor trauma patients often notice a significant reddening and blistering around the wound for up to 48 hours later ( hyperreactivity of the skin). This is a major criterion in the early detection of the disease. The so-called "cat's elbow test ", also called pathergy test, describes such excessive skin reaction with inflammation and nodules of the skin by a doctor injecting normal saline 0.5 ml intradermally. If there is reasonable suspicion immunological blood tests to determine the immunoglobulin composition are required.

Therapy

The treatment is done in the acute stage with corticosteroids (anti-inflammatory, anti-proliferative ), and in severe cases with frequent recurrences with immunosuppressants ( azathioprine, cyclosporine, chlorambucil, cyclophosphamide). If that does not lead to the desired result, a infliximab therapy may be prescribed. Also, thalidomide is named as a potential therapeutic agent.