Behçet's disease

Behçet's disease
Other names
  • Behçet disease
  • Behçet's syndrome
  • Morbus Behçet
  • Silk Road disease
A person with Behçet's disease exhibiting hypopyon, which can be seen in anterior uveitis
Pronunciation
  • /bɛˈɛt/, Turkish: [behˈtʃet]
SpecialtyRheumatology, Immunology
SymptomsMouth sores, genital sores, inflammation of the eye, arthritis,[1] chronic fatigue
ComplicationsBlindness, joint inflammation, blood clots, aneurysm[2]
Usual onset20s to 40s[1][2]
DurationLong term[1]
CausesUnknown[1]
Diagnostic methodBased on symptoms[1]
Differential diagnosisReactive arthritis, Stevens–Johnson syndrome, Sweet syndrome[2]
MedicationImmunosuppressive medication such as corticosteroids[1]
PrognosisOften improves with time[2]
FrequencyRare (US, EU), more common (Middle East, Asia)[2]

Behçet's disease (BD) is a type of inflammatory disorder which affects multiple parts of the body.[2] The most common symptoms include painful sores on the mucous membranes of the mouth and other parts of the body, inflammation of parts of the eye, and arthritis.[1][2] The sores can last from a few days, up to a week or more.[2] Less commonly there may be inflammation of the brain or spinal cord, blood clots, aneurysms, or blindness.[1][2] Often, the symptoms come and go.[1]

The cause is unknown.[1] It is believed to be partly genetic.[2] Behçet's is not contagious.[1] Diagnosis is based on at least three episodes of mouth sores in a year, together with at least two of the following: genital sores, eye inflammation, skin sores, a positive skin prick test.[1]

There is no cure.[1] Treatments may include immunosuppressive medication such as corticosteroids and anti-TNFs as well as lifestyle changes.[1] Lidocaine mouthwash may help with the pain.[2] Colchicine may decrease the frequency of attacks.[2]

While rare in the United States and Europe, it is more common in the Middle East and Asia.[2] In Turkey, for example, about 2 per 1,000 are affected.[2] Onset is usually in a person's twenties or forties.[1][2] The disease was initially described by Turkish dermatologist Hulusi Behçet in 1937.[3]

  1. ^ a b c d e f g h i j k l m n o Fleming, Ray (November 2014). "Fast Facts About Behçet's Disease". www.niams.nih.gov. Archived from the original on 13 May 2017. Retrieved 29 May 2017.
  2. ^ a b c d e f g h i j k l m n o "Behçet's Syndrome". NORD (National Organization for Rare Disorders). 2015. Archived from the original on 11 February 2017. Retrieved 29 May 2017.
  3. ^ Ball, Gene V.; Fessler, Barri J.; Bridges, S. Louis Jr. (2014). Oxford Textbook of Vasculitis (3 ed.). OUP Oxford. p. 491. ISBN 9780191667022. Archived from the original on 10 September 2017.