The pathergy test has a specificity of 95 percent to 100 percent, but the results are often negative in American and European patients
skin lesions (papulo-pustules, folliculitis, erythema nodosum, acne in post-adolescents not on corticosteroids)Despite the inclusive criteria set forth by the International Study Group, there are cases where not all the criteria can be met and therefore a diagnosis cannot readily be made. There is however a set of clinical findings that a physician can rely upon in making a tentative diagnosis of the disease; essentially Behçets disease does not always follow the International Study Group guidelines and so a high degree of suspicion for a patient who presents having any number of the following findings is necessary:
arthritis/arthralgia
cardio-vascular problems of an inflammatory origin
changes of personality, psychoses
deep vein thrombosis
epididymitis
extreme exhaustion - chronic fatigue
inflammatory problems in chest and lungs
mouth ulcers
nervous system symptoms
problems with hearing or balance
stomach or bowel inflammation
superficial thrombophlebitis
any other members of the family with a diagnosis of Behçets disease. Treatment
Current treatment is aimed at easing the symptoms, reducing inflammation, and controlling the immune system. The quality of the evidence for treating the oral ulcers associated with Behçets disease, however, is poor.High-dose corticosteroid therapy is often used for severe disease manifestations. Anti-TNF therapy such as infliximab has shown promise in treating the uveitis associated with the disease. Another Anti-TNF agent, etanercept, may be useful in people with mainly skin and mucosal symptoms.
However, B51 tends not to be found in disease when a certain SUMO4 gene variant is involved, and symptoms appear to be milder when HLA-B27 is present. At the current time, a similar infectious origin has not yet been confirmed that leads to Behçets disease, but certain strains of S. sanguinis has been found to have a homologous antigenicity.Vasculitis resulting in occlusion of the vessels supplying the optic nerve may be the cause of acute optic neuropathy and progressive optic atrophy in Behçets disease. Histological evaluation in a reported case of acute optic neuropathy demonstrated substitution of the axonal portion of the optic nerve with fibrous astrocytes without retinal changes. CNS involvement in Behçets disease may lead to intracranial hypertension most commonly due to dural venous sinus thrombosis and subsequent secondary optic atrophy. Diagnosis
There is no specific pathological testing or technique available for the diagnosis of the disease, although the International Study Group criteria for the disease are highly sensitive and specific, involving clinical criteria and a pathergy test. Behçets disease has a high degree of resemblance to diseases that cause mucocutaneous lesions such as Herpes simplex labialis, and therefore clinical suspicion should be maintained until all the common causes of oral lesions are ruled out from the differential diagnosis.Visual acuity, or color vision loss with concurrent mucocutaneous lesions or systemic Behçets disease symptoms should raise suspicion of optic nerve involvement in Behçets disease and prompt a work-up for Behçets disease if not previously diagnosed in addition to an ocular work-up.