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+#REDIRECT [[Adamantiades-Behçet's-disease]]
-{{Distinguish|Becker disease}}
-{{Infobox disease
- | Name            = Behçet's disease
- | Image           = Behcets disease.jpg
- | Caption         = Behçet's mouth ulcer
- | DiseasesDB      = 1285
- | ICD10           = {{ICD10|M|35|2|m|30}}
- | ICD9            = {{ICD9|136.1}}
- | ICDO            =
- | OMIM            = 109650
- | MedlinePlus     =
- | eMedicineSubj   = med
- | eMedicineTopic  = 218
- | eMedicine_mult  = {{eMedicine2|ped|219}} {{eMedicine2|derm|49}} {{eMedicine2|oph|425}}
- | MeshID          = D001528
-}}
-'''Behçet's disease''', sometimes called '''Behçet's syndrome''', '''Morbus Behçet''', or '''Silk Road disease''', is a rare immune-mediated  [[systemic]] [[vasculitis]]<ref name="urlGlossary, ">{{cite web |url=http://medicalcenter.osu.edu/patientcare/healthcare_services/multiple_sclerosis/ms_glossary/Pages/index.aspx |title=Glossary, |work= |accessdate=2009-03-28}}</ref> that often presents with mucous membrane ulceration and ocular involvements. Behçet's disease (BD) was named in 1937 after the Turkish dermatologist Hulusi Behçet, who first described the triple-symptom complex of recurrent oral aphthous ulcers, genital ulcers, and [[uveitis]]. Behçet's original article in German together with an English translation and a brief modern commentary are available on-line<ref>{{cite journal |author=Viggor SF, Willis AM, Jawad ASM |title=Behçet's disease |journal=Grand Rounds |volume=11 |pages=L1-L2 |year=2011 |doi=10.1102/1470-5206.2011.L001 |url=http://www.grandrounds-e-med.com/articles/gr11l001}}</ref>. As a systemic disease, it can also involve [[visceral organs]] such as the gastrointestinal tract, pulmonary, musculoskeletal, and neurological systems. This syndrome can be fatal, due to ruptured vascular [[aneurysms]], or severe neurological complications.<ref name=mk>American College of Physicians (ACP). <u>Medical Knowledge Self-Assessment Program (MKSAP-15): Rheumatology</u>. "Systemic vasculitis - Behcet disease." p. 71-73. ISBN 978-1-934465-30-1</ref>
-==Signs & Symptoms==
-===Integumentary system (Skin and mucosa)===
-Nearly all patients present with some form of painful oral mucocutaneous ulcerations in the form of [[aphthous ulcer]]s or non-scarring oral lesions.<ref name=mk /> The oral lesions are similar to those found in [[inflammatory bowel disease]] and can be relapsing.<ref name=mk /> Painful genital ulcerations usually develop around the anus, vulva or scrotum and cause scarring in 75% of the patients.<ref name=mk /> Additionally, patients may present with [[erythema nodosum]], cutaneous pustular vasculitis, and lesions similar to [[pyoderma gangrenosum]].<ref name=mk />
-===Ocular system===
-[[File:Hypopyon.jpg|Right|thumb|A patient depicting hypopyon which can be seen in [[anterior uveitis]] in a patient with Behcet's disease.]]
-Inflammatory eye disease can develop early in the disease course and lead to permanent vision loss in 20% of cases. Ocular involvement can be in the form of [[posterior uveitis]], [[anterior uveitis]], or [[retinal vasculitis]]. Anterior uveitis presents with painful eyes, conjuctival redness, [[hypopyon]], and decreased visual acuity, while posterior uveitis presents with painless decreased visual acuity and visual field floaters. A rare form of ocular (eye) involvement in this syndrome is retinal vasculitis which presents with painless decrease of vision with the possibility of floaters or visual field defects.<ref name={zakir shah}
-====Optic Nerve====
-Optic nerve involvement in Behçet's disease is rare, typically presenting as progressive  [[optic atrophy]] and visual loss. However, cases of acute optic neuropathy (specifically [[anterior ischemic optic neuropathy]]) have also been reported to occur.<ref>{{cite web|author=Eye |url=http://www.nature.com/eye/journal/v25/n3/full/eye2010208a.html |title=Access : A case of anterior ischemic optic neuropathy associated with Behcet's disease : Eye |publisher=Nature.com |date=2011-01-07 |accessdate=2011-08-03}}</ref> Optic nerve atrophy has been identified as the most common cause of visual impairment. Behçet's disease may result in primary or secondary optic nerve involvement. Papilledema as a result of [[dural sinus thrombosis]]<ref name="Fujikado"/> and atrophy resulting from retinal disease, have been characterized as secondary causes of optic nerve atrophy in Behçet's disease.<ref name="Ozdal"/><ref name="Kansu"/>
-[[Image:eye dilate.gif|thumb|left|alt=A Animation that illustrates the pupillary light reflex. When the light is turned on, the pupil reacts by constricting.|''[[Pupillary light reflex]]''.]]
-Signs and symptoms of acute [[optic neuropathy]] include painless loss of vision which may affect either one or both eyes, reduced visual acuity, reduced color vision, [[relative afferent pupillary defect]], central [[scotoma]], swollen optic disc, [[macular edema]], or retrobulbar pain. When these symptoms occur with concurrent mucocutaneous ulcerations, they raise suspicion of acute optic neuropathy in Behçet's Disease. Progressive optic atrophy may result in decreased visual acuity or color vision. [[Intracranial hypertension]] with [[papilledema]] may be present.
-[[Image:Eyevessels 001.jpg|thumb|left|alt=A left eye vessels|''Funduscopic photo left eye centered on the [[optic disc]]''.]]
-===Gastrointestinal tract (bowels)===
-GI manifestations include abdominal pain, nausea, and diarrhea with or without blood, and they often involve the ileocecal valve.<ref name=mk /> Many patients with BD often complain about abdominal tenderness, bloating and generic abdominal discomfort that closely mimicks irritable bowel syndrome.
-===Pulmonary (lungs)===
-Lung involvement is typically in the form of [[hemoptysis]], [[pleuritis]], cough, or fever, and in severe cases can be life threatening if the outlet pulmonary artery develops an aneurysm which ruptures causing severe vascular collapse and death from bleeding in the lungs.<ref name=mk />
-===Musculoskeletal system (muscle, joint)===
-[[Arthralgia]] is seen in up to half of patients, and is usually a non-erosive poly or oligoarthritis primarily of the large joints of the lower extremities.<ref name=mk />
-===Neurological system===
-Neurological involvements range from [[aseptic meningitis]] to vascular thrombosis such as [[dural sinus thrombosis]] and [[organic brain syndrome]] manifesting with confusion, seizures, and memory loss.<ref name=mk /> They often appear late in the progression of the disease but are associated with a poor prognosis.
-{{-}}
-==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.<ref name=mk /><ref name="pmid1970380">{{cite journal |author= International Study Group for Behçet's Disease |title=Criteria for diagnosis of Behçet's disease |journal=Lancet |volume=335 |issue=8697 |pages=1078–80 |year=1990 |month=May |pmid=1970380 |doi= 10.1016/0140-6736(90)92643-V|url=}}</ref> Behçet's 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 and/or systemic Behçet's symptoms should raise suspicion of optic nerve involvement in Behçet's disease and prompt a work-up for Behçet's disease if not previously diagnosed in addition to an ocular work-up. Diagnosis of Behçet's disease is based on clinical findings including oral and genital ulcers, skin lesions such as erythema nodosum, acne, or folliculitis, ocular inflammatory findings and a pathergy reaction. Inflammatory markers such ESR, and CRP may be elevated. A complete ophthalmic examination may include a slit lamp examination, [[optical coherence tomography]] to detect nerve loss, visual field examinations, fundoscopic examination to assess optic disc atrophy and retinal disease, fundoscopic angiography, and visual evoked potentials, which may demonstrate increased latency. Optic nerve enhancement may be identified on Magnetic Resonance Imaging (MRI) in some patients with acute optic neuropathy. However, a normal study does not rule out optic neuropathy. Cerebrospinal fluid (CSF) analysis may demonstrate elevated protein level with or without pleocytosis. Imaging including angiography may be indicated to identify dural venous sinus thrombosis as a cause of intracranial hypertension and optic atrophy.
-===International Study Group diagnostic guidelines===
-[[image:Behcet.JPG|thumb|right|Magnetic resonance venogram demonstrating occlusion of the left sigmoid and transverse sinuses.]]
-According to the International Study Group guidelines, for a patient to be diagnosed with Behçet's disease,<ref name="pmid1970380">{{cite journal
-|author=
-|title=Criteria for diagnosis of Behçet's disease. International Study  Group for Behçet's Disease
-|journal=Lancet
-|volume=335
-|issue=8697
-|pages=1078–80
-|year=1990
-|month=May
-|pmid=1970380
-|doi=10.1016/0140-6736(90)92643-V
-|last1=Internationalstudygroupforbehc
-}}</ref> the patient must have oral ([[aphthous]]) ulcers (any shape, size or number at least 3 times in any 12 months period) along with 2 out of the following 4 "[[hallmark]]" symptoms:
-*genital ulcers (including [[anus|anal]] ulcers and spots in the genital region and swollen [[testicle]]s or [[epididymitis]] in men)
-*[[skin]] [[lesion]]s (papulo-pustules, [[folliculitis]], [[erythema nodosum]], [[acne]] in post-adolescents not on corticosteroids)
-*[[human eye|eye]] inflammation ([[iritis]], [[uveitis]], retinal vasculitis, cells in the vitreous)
-*[[pathergy reaction]] (papule >2&nbsp;mm dia. 24-48 hrs or more after needle-prick). The pathery test has a specificity of 95% to 100%, but the results are often negative in American and European patients<ref name=mk />
-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 Behçet's 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:
-*[[mouth]] [[sore|ulcers]]
-*[[arthritis]]/[[arthralgia]]
-*[[nervous system]] symptoms
-*[[stomach]] and/or [[bowel]] inflammation
-*deep [[vein]] [[thrombosis]]
-*superficial [[thrombophlebitis]]
-*[[epididymitis]]
-*cardio-vascular problems of an inflammatory origin
-*inflammatory problems in [[chest]] and [[lungs]]
-*problems with hearing and/or balance
-*extreme exhaustion
-*changes of [[wikt:personality|personality]], [[psychoses]]
-*any other members of the family with a diagnosis of Behçet disease.
-==Pathogenesis==
-The [[etiology]] is not well-defined, but it is primarily characterized by auto-inflammation of the blood vessels. Although sometimes erroneously referred to as a "diagnosis of exclusion," the diagnosis can sometimes be reached by pathologic examination of the affected areas.<ref name="urlBehcet Disease: Overview - eMedicine Dermatology">{{cite web |url=http://emedicine.medscape.com/article/1122381-overview |title=Behcet Disease: Overview - eMedicine Dermatology |work= |accessdate=2009-03-28}}</ref>
-The primary mechanism of the damage is an overactive immune system that seems to target the patient's own body. The primary cause is not well known. In fact, as of now, no one knows why the immune system starts to behave this way in Behçet's disease. There does however seem to be a genetic component involved, as first degree relatives of the affected patients are often affected in more than expected proportion for the general population.
-==Treatment==
-Current treatment is aimed at easing the symptoms, reducing inflammation, and controlling the immune system. High dose Corticosteroid therapy (1&nbsp;mg/kg/d oral prednisone) is indicated for severe disease manifestations.<ref>CMDT (Current Medical Diagnosis & Treatment) 2007, Chapter 20, page 872</ref> [[TNF inhibitor|Anti-TNF]] therapy such as [[infliximab]] has shown promise in treating the uveitis associated with the disease.<ref>{{cite journal | author=Sfikakis PP, Theodossiadis PG, Katsiari CG, Kaklamanis P, Markomichelakis NN | title=Effect of infliximab on sight-threatening panuveitis in Behcet's disease | journal=Lancet | year=2001 | pages=295–6 | volume=358 | issue=9278  | pmid=11498218 | doi=10.1016/S0140-6736(01)05497-6}}</ref><ref>{{cite journal | author=Sfikakis PP | title=Behçet's disease: a new target for anti-tumour necrosis factor treatment | journal=Ann Rheum Dis | year=2002 | pages=ii51–3 | volume=61 Suppl 2  | pmid=12379622 | pmc=1766720 | doi=10.1136/ard.61.suppl_2.ii51 | issue=Suppl 2}}</ref> Another Anti-TNF agent, [[Etanercept]], may be useful in patients with mainly skin and mucosal symptoms.<ref>{{cite journal | author=Melikoglu M, Fresko I, Mat C, Ozyazgan Y, Gogus F, Yurdakul S, Hamuryudan V, Yazici H | title=Short-term trial of etanercept in Behcet's disease: a double blind, placebo controlled study | journal=J Rheumatol | year=2005 | pages=98–105 | volume=32 | issue=1  | pmid=15630733}}</ref>
-Interferon alfa-2a may also be an effective alternative treatment, particularly for the genital and oral ulcers<ref>{{cite journal | author=Alpsoy E, Durusoy C, Yilmaz E, Ozgurel Y, Ermis O, Yazar S, Basaran E | title=Interferon alfa-2a in the treatment of Behcet disease: a randomized placebo-controlled and double-blind study | journal=Arch Dermatol | year=2002 | pages=467–71 | volume=138 | issue=4  | pmid=11939808 | doi=10.1001/archderm.138.4.467}}</ref> as well as ocular lesions.<ref>{{cite journal | author=Kotter I, Zierhut M, Eckstein AK, Vonthein R, Ness T, Gunaydin I, Grimbacher B, Blaschke S, Meyer-Riemann W, Peter HH, Stubiger N | title=Human recombinant interferon alfa-2a for the treatment of Behçet's disease with sight threatening posterior or panuveitis | journal=Br J Ophthalmol | year=2003 | pages=423–31 | volume=87 | issue=4  | pmid=12642304 | doi=10.1136/bjo.87.4.423 | pmc=1771623}}</ref> [[Azathioprine]], when used in combination with interferon alfa-2b also shows promise,<ref>{{cite journal | author=Hamuryudan V, Ozyazgan Y, Fresko Y, Mat C, Yurdakul S, Yazici H | title=Interferon alfa combined with azathioprine for the uveitis of Behcet's disease: an open study | journal=Isr Med Assoc J | year=2002 | pages=928–30 | volume=4 | issue=11 Suppl  | pmid=12455182}}</ref> and [[Colchicine]] can be useful for treating some genital ulcers, [[erythema nodosum]], and arthritis.<ref>{{cite journal | author=Yurdakul S, Mat C, Tuzun Y, Ozyazgan Y, Hamuryudan V, Uysal O, Senocak M, Yazici H | title=A double-blind trial of colchicine in Behcet's syndrome | journal=Arthritis Rheum | year=2001 | pages=2686–92 | volume=44 | issue=11  | pmid=11710724 | doi=10.1002/1529-0131(200111)44:11<2686::AID-ART448>3.0.CO;2-H}}</ref>
-[[Thalidomide]] has also been used due to its immune-modifying effect.<ref>{{cite journal | author=Hamuryudan V, Mat C, Saip S, Ozyazgan Y, Siva A, Yurdakul S, Zwingenberger K, Yazici H | title=Thalidomide in the treatment of the mucocutaneous lesions of the Behcet syndrome. A randomized, double-blind, placebo-controlled trial | journal=Ann Intern Med | year=1998 | pages=443–50 | volume=128 | issue=6  | pmid=9499327}}</ref> [[Dapsone]] and [[rebamipide]] have been shown, in small studies, to have beneficial results for mucocutaneous lesions.<ref>{{cite journal | author=Matsuda T, Ohno S, Hirohata S, Miyanaga Y, Ujihara H, Inaba G, Nakamura S, Tanaka S, Kogure M, Mizushima Y | title=Efficacy of rebamipide as adjunctive therapy in the treatment of recurrent oral aphthous ulcers in patients with Behcet's disease: a randomised, double-blind, placebo-controlled study | journal=Drugs R D | year=2003 | pages=19–28 | volume=4 | issue=1  | pmid=12568631 | doi=10.2165/00126839-200304010-00002}}</ref><ref>{{cite journal | author=Sharquie KE, Najim RA, Abu-Raghif AR | title=Dapsone in Behcet's disease: a double-blind, placebo-controlled, cross-over study | journal=J Dermatol | year=2002 | pages=267–79 | volume=29 | issue=5  | pmid=12081158}}</ref>
-Given its rarity, the optimal treatment for acute optic neuropathy in Behçet's disease has not been established. Early identification and treatment is 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.<ref name="Voros" /> [[Immunosuppressant]]s such as interferon alpha and tumour necrosis factor antagonists may improve though not completely reverse symptoms of ocular Behçet's, 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.
-==Pathophysiology==
-[[Image:HLA-B*5101.png|thumb|150 px|right|[[HLA-B51]] is strongly associated with Behçet's disease<ref>{{Cite journal| volume = 100 | issue = 9 | pages = 1455–1458 | last = Ohno | first = S. | coauthors = M. Ohguchi, S. Hirose, H. Matsuda, A. Wakisaka, M. Aizawa | title = Close association of HLA-Bw51 with Behcet's disease | journal = Archives of Ophthalmology
-| year = 1982| pmid = 6956266}}</ref>]]
-Behçet's disease is considered more prevalent in the areas surrounding the [[Silk Road|old silk trading routes]] in the [[Middle East]] and in [[Central Asia]]. Thus, it is sometimes known as ''Silk Road Disease''.  However, this disease is not restricted to people from these regions. A large number of serological studies show a linkage between the disease and [[HLA-B51]].<ref name="pmid18214795">{{cite journal |author=Durrani K, Papaliodis GN |title=The genetics of Adamantiades-Behcet's disease |journal=Semin Ophthalmol |volume=23 |issue=1 |pages=73–9 |year=2008 |pmid=18214795 |doi=10.1080/08820530701745264 }}</ref> HLA-B51 is more frequently found from the Middle East to South Eastern Siberia, but the incidence of B51 in some studies was 3 fold higher than the normal population. However, B51 tends not to be found in disease when a certain [[SUMO4]] gene variant is involved,<ref name="pmid18657476">{{cite journal |author=Hou S |title=SUMO4 gene polymorphisms in Chinese Han patients with Behcet's disease |journal=Clin. Immunol. |volume= 129|issue= 1|pages= 170–5|year=2008 |month=July |pmid=18657476 |doi=10.1016/j.clim.2008.06.006 |author-separator=, |author2=Yang P |author3=Du L |display-authors=3 |last4=Zhou |first4=Hongyan |last5=Lin |first5=Xiaomin |last6=Liu |first6=Xiaoli |last7=Kijlstra |first7=Aize }}</ref> and symptoms appear to be milder when [[HLA-B27]] is present.<ref name="pmid17923546">{{cite journal |author=Ahn JK, Park YG |title=Human leukocyte antigen B27 and B51 double-positive Behçet uveitis |journal=Arch. Ophthalmol. |volume=125 |issue=10 |pages=1375–80 |year=2007 |month=October |pmid=17923546 |doi=10.1001/archopht.125.10.1375 }}</ref>  At the current time, a similar infectious origin has not yet been confirmed that leads to Behçet's disease, but certain strains of ''[[Streptococcus sanguinis]]'' has been found to have a homologous antigenicity.<ref name="pmid16514412">{{cite journal |author=Yanagihori H, Oyama N, Nakamura K, Mizuki N, Oguma K, Kaneko F |title=Role of IL-12B promoter polymorphism in Adamantiades-Behcet's disease susceptibility: An involvement of Th1 immunoreactivity against Streptococcus Sanguinis antigen |journal=J. Invest. Dermatol. |volume=126 |issue=7 |pages=1534–40 |year=2006 |month=July |pmid=16514412 |doi=10.1038/sj.jid.5700203 }}</ref>
-[[Vasculitis]] resulting in occlusion of the vessels supplying the optic nerve may be the cause of acute optic neuropathy and progressive optic atrophy in Behçet's 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.<ref name="Kansu"/> CNS involvement in Behçet's may lead to intracranial hypertension most commonly due to dural venous sinus thrombosis<ref name="Fujikado"/> [8-10] and subsequent secondary optic atrophy.
-==Epidemiology==
-The syndrome is rare in the United States, but is common in the Middle East and Asia, suggesting a possible cause endemic to the tropical areas.<ref>[http://www.nlm.nih.gov/medlineplus/behcetssyndrome.html Behcet's syndrome (Medline Plus).]</ref> It is not associated with [[cancer]], and links with tissue-types (which are under investigation) are not certain. It also does not follow the usual pattern for [[autoimmune diseases]].  However, one study has revealed a possible connection to food allergies, particularly to dairy products.<ref>{{cite journal | doi = 10.1136/ard.61.5.459 | author = Triolo | year = 2002 | title = Humoral and cell mediated immune response to cow's milk proteins in Behçet's disease | url = | journal = Ann Rheum Dis | volume = 61 | issue = 5| pages = 459–62 | pmid = 11959773 | pmc = 1754076 | display-authors = 1 | last2 = Accardo-Palumbo | first2 = A | last3 = Dieli | first3 = F | last4 = Ciccia | first4 = F | last5 = Ferrante | first5 = A | last6 = Giardina | first6 = E | last7 = Licata | first7 = G }}</ref> 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.<ref>[http://www.nhs.uk/Conditions/Behcets-disease/Pages/introduction.aspx?url=Pages/what-is-it.aspx Behcet's disease.]</ref> Globally, males are affected more frequently than females.<ref name="pmid16476027">{{cite journal |author=Escudier M, Bagan J, Scully C |title=Number VII Behçet's disease (Adamantiades syndrome) |journal=Oral Dis |volume=12 |issue=2 |pages=78–84 |year=2006 |month=March |pmid=16476027 |doi=10.1111/j.1601-0825.2005.01144.x |url=}}</ref> In the United States, more females are affected than males.{{Citation needed|date=August 2008}}
-In an epidemiologic study by Krause et al., 56% of patient’s 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% of patients.] Ocular Behçet's 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% and 7.4%, respectively. Other fundoscopic findings include vascular sheathing(23.7%),<ref name="Ozdal"/> retinal hemorrhage(9%),<ref name="Ozdal"/> macular edema(11.3%),<ref name="Ozdal"/> branch retinal vein occlusion(5.8%),<ref name="Ozdal"/> and retinal edema(6.6%).<ref name="Ozdal"/> However, optic atrophy was the most significant cause of visual impairment identified in 54% of patients with ocular Behçet's disease and permanent visual impairment.<ref name="Ozdal"/>
-==History==
-Behçet disease is [[eponym|named]] after [[Hulusi Behçet]] (1889–1948), the [[Turkish people|Turkish]] [[dermatologist]] and [[scientist]] who first recognized 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.<ref name="WhoNamedIt">{{WhoNamedIt|synd|1863}}</ref><ref>H. Behçet. Über rezidivierende, aphtöse, durch ein Virus verursachte Geschwüre am Mund, am Auge und an den Genitalien.
-Dermatologische Wochenschrift, Hamburg, 1937, 105(36): 1152-1163.</ref>  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 3rd Epidemion-book.<ref>Johns Hopkins Vasculitis Center (2004). [http://vasculitis.med.jhu.edu/typesof/behcets.html Johns Hopkins Vasculitis Center Discusses Behcets Disease]. Retrieved September 9, 2005.</ref> Its first modern formal description was published in 1922.<ref name="WhoNamedIt" />
-Some sources use the term "Adamantiades’ syndrome" or "Adamandiades-Behçet syndrome", for the work done by [[Benediktos Adamantiades]].<ref>B. Adamandiades. Sur un cas d'iritis à hypopyon récidivant. Annales d'oculistique, Paris, 1931, 168: 271-278.</ref> However, the current [[World Health Organization]]/[[ICD-10]] standard is "Behçet's disease". In 1991, [[Saudi Arabia]]n medical researchers described "neuro-Behçet's disease",<ref>{{cite journal | doi = 10.1111/j.1474-7766.2004.03-225.x | author = Malhotra Ravi | year = 2004 | title = Saudi Arabia | url = | journal = Practical Neurology | volume = 4 | issue = 3| pages = 184–185 }}</ref> a [[Neurology|neurological]] involvement in Behçet's disease, considered one of the most devastating manifestations of the disease as investigated by an Egyptian researcher [[Sahar Saleem]].<ref>S. Saleem (2005), [http://www.neurographics.org/4/2/1/4.shtml Neuro-Behçet's Disease: NBD], ''Neurographics'', Vol. 4, Issue 2, Article 1.</ref> The mechanism can be immune-mediated, or thrombotic.<ref name="pmid19161910">{{cite journal |author=Al-Araji A, Kidd DP |title=Neuro-Behçet's disease: epidemiology, clinical characteristics, and management |journal=Lancet Neurol |volume=8 |issue=2 |pages=192–204 |year=2009 |month=February |pmid=19161910 |doi=10.1016/S1474-4422(09)70015-8 |url=http://linkinghub.elsevier.com/retrieve/pii/S1474-4422(09)70015-8}}</ref> The term dates back to at least 1990.<ref name="pmid2188002">{{cite journal |author=Su SL, Way LJ, Lin RT, Peng MJ, Wu SC |title=Neuro-Behçet's disease: report of three cases with a review of the literature |journal=Gaoxiong Yi Xue Ke Xue Za Zhi |volume=6 |issue=3 |pages=155–62 |year=1990 |month=March |pmid=2188002 |doi= |url=}}</ref>
-== See also ==
-* [[Behcet's Disease of the Optic Nerve]]
-* [[List of cutaneous conditions]]
-==References==
-{{reflist|30em|refs=
-<ref name="Ozdal">{{Cite journal | last=Ozdal | first=PC | coauthors=Ortaç S, Taşkintuna I, Firat E | title=Posterior segment involvement in ocular Behçet's disease | journal=European Journal of Ophthalmology | volume=12 | issue=5 | pages=424–431 | month=Sep-Oct | year=2002 | pmid=12474927 }}</ref>
-<ref name="Fujikado">{{Cite journal | last=Fujikado | first=T | coauthors=Imagawa K | title=Dural sinus thrombosis in Behçet's disease&mdash;a case report | journal=Japanese Journal of Ophthalmology | volume=38 | issue=4 | pages=411–416 | year=1994 | pmid=7723211 }}</ref>
-<ref name="Kansu">{{Cite journal | last=Kansu | first=T | coauthors=Kirkali P, Kansu E, Zileli T | title=Optic neuropathy in Behçet's disease | journal=Journal of Clinical Neuro-ophthalmology | volume=9 | issue=4 | pages=277–280 | month=December | year=1989 | pmid=2531168 }}</ref>
-<ref name="Voros">Voros, G.M., S.S. Sandhu, and R. Pandit, Acute optic neuropathy in patients with Behcet's disease. Report of two cases. Ophthalmologica, 2006. 220(6): p.&nbsp;400-5.</ref>
-}}
-==Further reading==
-*Yamauchi, Y., et al., Suspected simultaneous bilateral anterior ischemic optic neuropathy in a patient with Behcet's disease. Ocul Immunol Inflamm, 2005. 13(4): p.&nbsp;317-25.
-*Krause, L., et al., Ocular involvement in Adamantiades-Behcet's disease in Berlin, Germany. Graefes Arch Clin Exp Ophthalmol, 2009. 247(5): p.&nbsp;661-6.
-*Brissaud, P., et al., Digital angiography for the diagnosis of dural sinus thrombosis in Behcet's disease. Arthritis Rheum, 1985. 28(3): p.&nbsp;359-60.
-*el-Ramahi, K.M. and M.Z. al-Kawi, Papilloedema in Behcet's disease: value of MRI in diagnosis of dural sinus thrombosis. J Neurol Neurosurg Psychiatry, 1991. 54(9): p.&nbsp;826-9.
-*Fujikado, T. and K. Imagawa, Dural sinus thrombosis in Behcet's disease—a case report. Jpn J Ophthalmol, 1994. 38(4): p.&nbsp;411-6.
-==External links==
-* {{DMOZ|Health/Conditions_and_Diseases/Musculoskeletal_Disorders/Connective_Tissue/Behcet's_Syndrome/}}
-{{Systemic connective tissue disorders}}
-{{DEFAULTSORT:Behcet's Disease}}
-[[Category:Autoimmune diseases]]
-[[Category:Conditions of the mucous membranes]]
-[[Category:Inflammations]]
-[[Category:Systemic connective tissue disorders]]
-[[Category:Rare diseases]]
-[[ar:مرض بهجت]]
-[[ca:Malaltia de Behçet]]
-[[de:Morbus Adamantiades-Behçet]]
-[[es:Síndrome de Behcet]]
-[[fa:بیماری راه ابریشم]]
-[[fr:Maladie de Behçet]]
-[[it:Sindrome di Behçet]]
-[[he:מחלת בכצ'ט]]
-[[kk:Бехчета ауруы]]
-[[nl:Ziekte van Behçet]]
-[[ja:ベーチェット病]]
-[[pl:Choroba Behçeta]]
-[[pt:Doença de Behçet]]
-[[ru:Болезнь Бехчета]]
-[[sr:Бехчетов синдром]]
-[[fi:Behçetin tauti]]
-[[tr:Behçet hastalığı]]
-[[uk:Синдром Бехчета]]
-[[vi:Bệnh Behçet]]
-[[zh:貝賽特氏症]]