Behcet's Disease
The M. Behçet is a chronic, recurrent inflammation of the classic triad of iritis (eye inflammation) and ulcerative mucosal lesions (ulcers) in the mouth and on the sexual organs.
The origin of the M. Behçet is as yet unclear.
A connection to genetic factors is described (HLA-B5-genotype). A family history is. Infections are assumed to be possible triggers.
The disease is expressed in a presumably by immune complexes mediated inflammation of small blood vessels (vasculitis), primarily of the venous system and deposition of inflammatory proteins and autoantibodies against the oral mucosa.
Frequency
The M. Behçet is an extremely rare disease of young adults ages in Europe. In the eastern Mediterranean countries and in Japan it is more often found. In Turkey, a prevalence (frequency) was found up to 19/5131 population> 10 years. Males are affected slightly more often.
Symptoms
- Painful Mucosal ulcers (aphthous ulcers) with a diameter of 3-10 mm.
- Ocular involvement with uveitis (inflammation of the eye chamber vordernen) with a hypopyon (accumulation of pus on the floor of the anterior chamber of the eye) and risk of blindness.
- Joint pain in hands, knees and ankle joints (arthralgia) in the M. Behcet are usually mild and had its origin in a synovitis.
- The involvement of other vessels is approximately 35% of patients and manifests itself among others in deep and superficial vein thrombosis, sinus thrombosis (cerebral veins), and rarely with involvement of arteries in the formation of aneurysms (vasodilation).
- When other symptoms may occur as part of the M. Behçet arthralgia, gastrointestinal lesions, thrombophlebitis, neurological manifestation.
- The central nervous system is affected in about 20% of cases. Aseptic meningitis, hemiparesis, loss of balance and changes in mood can be signs of a neurological manifestation.
Diagnosis
The diagnosis of M. Behçet is based on the characteristic symptoms. (Diagnostic criteria of the Behcet Syndrome Research Committee of Japan 1972)
- Recurring Mucosal ulcers in the mouth
- Skin lesions
- Erythema nodosum-like eruptions,
- Subcutaneous thrombophlebitis
- Hyperirritability the skin (Pathergy test) - Eye lesions
- Recurring hypopyoniritis or iridocyclitis
- Chorioretinitis - Genital ulcerations
Therapy
The treatment of M. Behçet consists of the administration of cortisone. In particular, the aphthous ulcers talk to the administration of thalidomide good. Furthermore, in the treatment and colchicine, cyclosporin A, are cyclophosphamide, azathioprine, chlorambucil, applied IFN-alpha and plasmapheresis.