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Behcet disease(BD)

MedGen UID:
2568
Concept ID:
C0004943
Disease or Syndrome
Synonyms: BD; Behcet Syndrome; Behcet's disease; Behcet's syndrome
SNOMED CT: Adamantiades-Behcet disease (310701003); Behcet syndrome (310701003); Behcet's syndrome (310701003); Behcet's disease (310701003)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
Concept ID:
C0600599
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
 
Monarch Initiative: MONDO:0007191
OMIM®: 109650
Orphanet: ORPHA117

Definition

Behçet disease is an inflammatory condition that affects many parts of the body. The health problems associated with Behçet disease result from widespread inflammation of blood vessels (vasculitis). This inflammation most commonly affects small blood vessels in the mouth, genitals, skin, and eyes.

Painful mouth sores called aphthous ulcers are usually the first sign of Behçet disease. These sores can occur on the lips, tongue, inside the cheeks, the roof of the mouth, the throat, and the tonsils. The ulcers look like common canker sores, and they typically heal within one to two weeks. About 75 percent of all people with Behçet disease develop similar ulcers on the genitals. These ulcers occur most frequently on the scrotum in men and on the labia in women.

Behçet disease can also cause painful bumps and sores on the skin. Most affected individuals develop pus-filled bumps that resemble acne. These bumps can occur anywhere on the body. Some affected people also have red, tender nodules called erythema nodosum. These nodules usually develop on the legs but can also occur on the arms, face, and neck.

An inflammation of the eye called uveitis is found in more than half of people with Behçet disease. Eye problems are more common in younger people with the disease and affect men more often than women. Uveitis can result in blurry vision and an extreme sensitivity to light (photophobia). Rarely, inflammation can also cause eye pain and redness. If untreated, the eye problems associated with Behçet disease can lead to blindness.

Joint involvement is also common in Behçet disease. Often this affects one joint at a time, with each affected joint becoming swollen and painful and then getting better.

Less commonly, Behçet disease can affect the brain and spinal cord (central nervous system), gastrointestinal tract, large blood vessels, heart, lungs, and kidneys. Central nervous system abnormalities can lead to headaches, confusion, personality changes, memory loss, impaired speech, and problems with balance and movement. Involvement of the gastrointestinal tract can lead to a hole in the wall of the intestine (intestinal perforation), which can cause serious infection and may be life-threatening.

The signs and symptoms of Behçet disease usually begin in a person's twenties or thirties, although they can appear at any age. Some affected people have relatively mild symptoms that are limited to sores in the mouth and on the genitals. Others have more severe symptoms affecting various parts of the body, including the eyes and the vital organs. The features of Behçet disease typically come and go over a period of months or years. In most affected individuals, the health problems associated with this disorder improve with age. [from MedlinePlus Genetics]

Clinical features

From HPO
Genital ulcers
MedGen UID:
57716
Concept ID:
C0151281
Disease or Syndrome
Decreased level of D-mannose in urine
MedGen UID:
1636806
Concept ID:
C4703626
Finding
A decrease in the level of D-mannose in the urine.
Raynaud phenomenon
MedGen UID:
20474
Concept ID:
C0034735
Disease or Syndrome
An episodic vasoconstriction resulting in discoloration of the skin and pain in the affected areas, often involving fingers or toes. Classically associated with triphasic color changes (white, blue, red) but may be biphasic. Often occurs in response to cold temperatures or emotional stress. May be primary or secondary to an underlying autoimmune disease.
Irritability
MedGen UID:
397841
Concept ID:
C2700617
Mental Process
A proneness to anger, i.e., a tendency to become easily bothered or annoyed.
Superficial thrombophlebitis
MedGen UID:
266934
Concept ID:
C1510431
Disease or Syndrome
Inflammation of a superficial vein associated with venous thrombosis (blood clot formation within the vein).
Arthritis
MedGen UID:
2043
Concept ID:
C0003864
Disease or Syndrome
Inflammation of a joint.
Epididymitis
MedGen UID:
4986
Concept ID:
C0014534
Disease or Syndrome
The presence of inflammation of the epididymis.
Erythema nodosum
MedGen UID:
41858
Concept ID:
C0014743
Disease or Syndrome
An erythematous eruption commonly associated with drug reactions or infection and characterized by inflammatory nodules that are usually tender, multiple, and bilateral.
Iridocyclitis
MedGen UID:
43961
Concept ID:
C0022073
Disease or Syndrome
A type of anterior uveitis, in which there is Inflammation of the iris and the ciliary body.
Oral ulcer
MedGen UID:
57699
Concept ID:
C0149745
Disease or Syndrome
Erosion of the mucous mebrane of the mouth with local excavation of the surface, resulting from the sloughing of inflammatory necrotic tissue.
Erythema
MedGen UID:
11999
Concept ID:
C0041834
Disease or Syndrome
Redness of the skin, caused by hyperemia of the capillaries in the lower layers of the skin.
Patchy alopecia
MedGen UID:
350774
Concept ID:
C1862862
Finding
Transient, non-scarring hair loss and preservation of the hair follicle located in in well-defined patches.
Chorioretinitis
MedGen UID:
942
Concept ID:
C0008513
Disease or Syndrome
An inflammation of the choroid and retina.
Hypopyon
MedGen UID:
43802
Concept ID:
C0020641
Disease or Syndrome
Presence of pus (appears as a white fluid) producing a fluid level in the inferior part of the anterior chamber.
Iritis
MedGen UID:
7160
Concept ID:
C0022081
Disease or Syndrome
Inflammation of the iris.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBehcet disease
Follow this link to review classifications for Behcet disease in Orphanet.

Professional guidelines

PubMed

Standardization of Uveitis Nomenclature (SUN) Working Group
Am J Ophthalmol 2021 Aug;228:80-88. Epub 2021 May 11 doi: 10.1016/j.ajo.2021.03.058. PMID: 33845008Free PMC Article
Cavalli G, Colafrancesco S, Emmi G, Imazio M, Lopalco G, Maggio MC, Sota J, Dinarello CA
Autoimmun Rev 2021 Mar;20(3):102763. Epub 2021 Jan 20 doi: 10.1016/j.autrev.2021.102763. PMID: 33482337
Saccucci M, Di Carlo G, Bossù M, Giovarruscio F, Salucci A, Polimeni A
J Immunol Res 2018;2018:6061825. Epub 2018 May 27 doi: 10.1155/2018/6061825. PMID: 29977929Free PMC Article

Recent clinical studies

Etiology

Standardization of Uveitis Nomenclature (SUN) Working Group
Am J Ophthalmol 2021 Aug;228:80-88. Epub 2021 May 11 doi: 10.1016/j.ajo.2021.03.058. PMID: 33845008Free PMC Article
Pérez-Garza DM, Chavez-Alvarez S, Ocampo-Candiani J, Gomez-Flores M
Am J Clin Dermatol 2021 May;22(3):367-378. Epub 2021 Mar 8 doi: 10.1007/s40257-021-00592-w. PMID: 33683567Free PMC Article
Tattevin P, Tchamgoué S, Belem A, Bénézit F, Pronier C, Revest M
Rev Neurol (Paris) 2019 Sep-Oct;175(7-8):475-480. Epub 2019 Jul 30 doi: 10.1016/j.neurol.2019.07.005. PMID: 31375286
Skef W, Hamilton MJ, Arayssi T
World J Gastroenterol 2015 Apr 7;21(13):3801-12. doi: 10.3748/wjg.v21.i13.3801. PMID: 25852265Free PMC Article
Akintoye SO, Greenberg MS
Dent Clin North Am 2014 Apr;58(2):281-97. Epub 2014 Jan 21 doi: 10.1016/j.cden.2013.12.002. PMID: 24655523Free PMC Article

Diagnosis

Tattevin P, Tchamgoué S, Belem A, Bénézit F, Pronier C, Revest M
Rev Neurol (Paris) 2019 Sep-Oct;175(7-8):475-480. Epub 2019 Jul 30 doi: 10.1016/j.neurol.2019.07.005. PMID: 31375286
Bulur I, Onder M
Clin Dermatol 2017 Sep-Oct;35(5):421-434. Epub 2017 Jun 27 doi: 10.1016/j.clindermatol.2017.06.004. PMID: 28916023
Wick MR
Semin Diagn Pathol 2017 May;34(3):261-272. Epub 2016 Dec 27 doi: 10.1053/j.semdp.2016.12.004. PMID: 28129926
Skef W, Hamilton MJ, Arayssi T
World J Gastroenterol 2015 Apr 7;21(13):3801-12. doi: 10.3748/wjg.v21.i13.3801. PMID: 25852265Free PMC Article
Akintoye SO, Greenberg MS
Dent Clin North Am 2014 Apr;58(2):281-97. Epub 2014 Jan 21 doi: 10.1016/j.cden.2013.12.002. PMID: 24655523Free PMC Article

Therapy

Emmi G, Bettiol A, Hatemi G, Prisco D
Lancet 2024 Mar 16;403(10431):1093-1108. Epub 2024 Feb 22 doi: 10.1016/S0140-6736(23)02629-6. PMID: 38402885
Pérez-Garza DM, Chavez-Alvarez S, Ocampo-Candiani J, Gomez-Flores M
Am J Clin Dermatol 2021 May;22(3):367-378. Epub 2021 Mar 8 doi: 10.1007/s40257-021-00592-w. PMID: 33683567Free PMC Article
Gasparyan AY, Ayvazyan L, Mukanova U, Yessirkepov M, Kitas GD
Ann Lab Med 2019 Jul;39(4):345-357. doi: 10.3343/alm.2019.39.4.345. PMID: 30809980Free PMC Article
Nelson CA, Stephen S, Ashchyan HJ, James WD, Micheletti RG, Rosenbach M
J Am Acad Dermatol 2018 Dec;79(6):987-1006. Epub 2018 Apr 11 doi: 10.1016/j.jaad.2017.11.064. PMID: 29653210
Saadoun D, Wechsler B
Orphanet J Rare Dis 2012 Apr 12;7:20. doi: 10.1186/1750-1172-7-20. PMID: 22497990Free PMC Article

Prognosis

Emmi G, Bettiol A, Hatemi G, Prisco D
Lancet 2024 Mar 16;403(10431):1093-1108. Epub 2024 Feb 22 doi: 10.1016/S0140-6736(23)02629-6. PMID: 38402885
Pérez-Garza DM, Chavez-Alvarez S, Ocampo-Candiani J, Gomez-Flores M
Am J Clin Dermatol 2021 May;22(3):367-378. Epub 2021 Mar 8 doi: 10.1007/s40257-021-00592-w. PMID: 33683567Free PMC Article
Gasparyan AY, Ayvazyan L, Mukanova U, Yessirkepov M, Kitas GD
Ann Lab Med 2019 Jul;39(4):345-357. doi: 10.3343/alm.2019.39.4.345. PMID: 30809980Free PMC Article
Skef W, Hamilton MJ, Arayssi T
World J Gastroenterol 2015 Apr 7;21(13):3801-12. doi: 10.3748/wjg.v21.i13.3801. PMID: 25852265Free PMC Article
Jubelt B, Mihai C, Li TM, Veerapaneni P
Curr Neurol Neurosci Rep 2011 Dec;11(6):543-52. doi: 10.1007/s11910-011-0228-5. PMID: 21956758

Clinical prediction guides

Emmi G, Bettiol A, Hatemi G, Prisco D
Lancet 2024 Mar 16;403(10431):1093-1108. Epub 2024 Feb 22 doi: 10.1016/S0140-6736(23)02629-6. PMID: 38402885
Wang C, Zhou W, Su G, Hu J, Yang P
Neurol Neuroimmunol Neuroinflamm 2022 Mar;9(2) Epub 2022 Jan 26 doi: 10.1212/NXI.0000000000001133. PMID: 35082168Free PMC Article
Sag E, Demir S, Ozen S
Curr Rheumatol Rep 2020 May 20;22(7):28. doi: 10.1007/s11926-020-00908-5. PMID: 32436006
Gasparyan AY, Ayvazyan L, Mukanova U, Yessirkepov M, Kitas GD
Ann Lab Med 2019 Jul;39(4):345-357. doi: 10.3343/alm.2019.39.4.345. PMID: 30809980Free PMC Article
Skef W, Hamilton MJ, Arayssi T
World J Gastroenterol 2015 Apr 7;21(13):3801-12. doi: 10.3748/wjg.v21.i13.3801. PMID: 25852265Free PMC Article

Recent systematic reviews

Muñoz SA, Kostianovsky A, Allievi A, Orden AO
Reumatol Clin (Engl Ed) 2023 Aug-Sep;19(7):386-391. doi: 10.1016/j.reumae.2022.12.005. PMID: 37661116
Chuang KW, Chang HC
Eur J Clin Invest 2022 Aug;52(8):e13778. Epub 2022 Apr 5 doi: 10.1111/eci.13778. PMID: 35342952
Berteau F, Rouviere B, Delluc A, Nau A, Le Berre R, Sarrabay G, Touitou I, de Moreuil C
Autoimmun Rev 2018 Aug;17(8):809-815. Epub 2018 Jun 8 doi: 10.1016/j.autrev.2018.02.012. PMID: 29890348
Al Bedah AM, Khalil MK, Posadzki P, Sohaibani I, Aboushanab TS, AlQaed M, Ali GI
J Altern Complement Med 2016 Oct;22(10):768-777. Epub 2016 Aug 24 doi: 10.1089/acm.2016.0193. PMID: 27557333
Merashli M, Ster IC, Ames PR
Semin Arthritis Rheum 2016 Feb;45(4):502-10. Epub 2015 Jul 4 doi: 10.1016/j.semarthrit.2015.06.018. PMID: 26239908

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