be useful in people with mainly skin and mucosal symptoms. apremilast may also be used to treat oral ulcers associated with behçet's disease. interferon alpha-2a may also be an effective alternative treatment, particularly for the genital and oral ulcers as well as ocular lesions. azathioprine, when used in combination with interferon alpha-2b also shows promise, and colchicine can be useful for treating some genital ulcers, erythema nodosum, and arthritis. benzathine‐penicillin may also reduce new arthritic attacks. thalidomide has also been used due to its immune-modifying effect. dapsone and rebamipide have been shown, in small studies, to have beneficial results for mucocutaneous lesions. given its rarity, the optimal treatment for acute optic neuropathy in behçet's disease has not been established. early identification and treatment are essential. response to ciclosporin, periocular triamcinolone, and iv methylprednisone followed by oral prednisone has been reported although relapses leading to irreversible visual loss may occur even with treatment. immunosuppressants such as interferon-alpha and tumour necrosis factor antagonists may improve though not completely reverse symptoms of ocular behçet's disease, which may progress over time despite treatment. when symptoms are limited to the anterior chamber of the eye prognosis is improved. posterior involvement, particularly optic nerve involvement, is a poor prognostic indicator. secondary optic nerve atrophy is frequently irreversible. lumbar puncture or surgical treatment may be required to prevent optic atrophy in cases of intracranial hypertension refractory to treatment with immunomodulators and steroids. ivig could be a treatment for severe or complicated cases. surgery surgical treatment of arterial manifestations of bd bears many pitfalls since the obliterative endarteritis of vasa vasorum causes thickening of the medial layer and splitting of elastin fibers. therefore, anastomotic pseudoaneurysms are likely to form, as well as pseudoaneurysms at the site of the puncture in case of angiography or endovascular treatment; furthermore, early graft occlusion may occur. for these reasons, invasive treatment should not be performed in the acute and active phases of the disease when inflammation is at its peak. the evaluation of disease's activity is usually based on relapsing symptoms, esr (erythrocyte sedimentation rate), and serum levels of crp (c‐reactive protein). endovascular treatment can be an effective and safe alternative to open surgery, with less postoperative complications, faster recovery time, and reduced need for intensive care, while offering patency rates and procedural success rates comparable with those of surgery. this notwithstanding, long‐term results of endovascular treatment in bd are still to be determined. epidemiology the syndrome is rare in the united states, africa and south america, but is common in asia, suggesting a possible cause endemic to those areas. a theory suggested that past exposure to lethal infectious agents might have fixed the genetic susceptibility factors to behçet's disease in those area. an estimated 15,000 to 20,000 americans have been diagnosed with this disease. in the uk, it is estimated to have about 1 case for every 100,000 people. globally, males are affected more frequently than females. in an epidemiologic study, 56 percent of patients with behçet's disease developed ocular involvement at a mean age of 30. ocular involvement was the first manifestation of behçet's disease in 8.6 percent of patients. ocular behçet's disease with involvement of the optic nerve is rarely reported. among patients with ocular behçet's disease funduscopic findings of optic atrophy, and optic disc paleness have been identified with a frequency of 17.9 percent and 7.4 percent, respectively. other fundoscopic findings include vascular sheathing (23.7%), retinal hemorrhage (9%), macular edema (11.3%), branch retinal vein occlusion (5.8%), and retinal edema (6.6%). however, optic atrophy was the most significant cause of visual impairment identified in 54 percent of patients with ocular behçet's disease and permanent visual impairment. the prevalence of this disease increases from north to south. it follows a more severe course in patients with an early age of onset particularly in patients with eye and gastrointestinal involvement. pregnancy with behçet's disease as a pre-existing disease in pregnancy or acquired, the pregnancy does not have an adverse effect on the course of behçet's disease and may possibly ameliorate its course. still, there is a substantial variability in clinical course between patients and even for different pregnancies in the same patient. also, the other way around, behçet's disease confers an increased risk of pregnancy complications, miscarriage and cesarean section. behçet's can cause male infertility, either as a result of the condition itself or of a side effect of concomitant medication such as colchicine, which is known to lower sperm count. history the first modern formal description of the symptoms was made by h. planner and f. remenovsky and published in 1922 in the archiv für dermatologie und syphilis. behçet's disease is named after hulusi behçet (1889–1948), the turkish dermatologist and scientist who first recognized the three main symptoms of the syndrome in one of his patients in 1924 and reported his research on the disease in journal of skin and venereal diseases in 1936. the name (morbus behçet) was formally adopted at the international congress of dermatology in geneva in september 1947. symptoms of this disease may have been described by hippocrates in the 5th century bc, in his epidemion (book 3, case 7). some sources use the term "adamantiades's syndrome" or "adamantiades–behçet syndrome", for the work done by benediktos adamantiades. however, the current world health organization/icd-10 standard is "behçet's disease". in 1991, saudi arabian medical researchers described neuro-behçet's disease, a neurological involvement in behçet's disease, considered one of the most devastating manifestations of the disease. the mechanism can be immune-mediated or thrombotic. the term dates back to at least 1990.