Behçet's disease is a multisystem inflammatory disease characterized by recurrent oral and genital ulcers. It was first described in 1937 and is most common along the Silk Road. Diagnosis relies on clinical criteria including oral ulcers plus at least two other symptoms like genital ulcers, eye inflammation, or skin lesions. Histologically there is neutrophilic and lymphocytic infiltration of blood vessels. Common features include oral, genital, eye, skin and joint involvement. Vascular thrombosis can also occur. Treatment focuses on reducing inflammation.

1. Presented by: Rasha Mohamed Alamien
Supervised by: Dr/ Alnoor Alaagib
10/12/15 1

2. 10/12/15 2

3. Background
Behçet's disease (BD):
First described in 1937 by Turkish dermatologist Hulusi
Behçet.
 Is a multisysemic inflammatory disease of unknown
etiology, characterized by relapsing episodes of oral
aphthous ulcers, genital ulcers, other skin lesions
(pathergy test), and ocular lesions.
Histologically, there is combinations of perivacsular
neutrophilic and lymphocytic infiltration, endothelial cell
damage or swelling coupled with fibrinoid necrosis and
thrombotic tendency10/12/15 3

4. Incidence and prevalence
Prevalence vary from region to region and from country to country.
Cases of BD seem to cluster along the ancient Silk Road, which
extends from eastern Asia to the Mediterranean basin .
Turkey: 80-370 in 100000
 It affects people mainly between the ages of 20 to 40.
 Both sexes are usually equally affected.
Male predominance in Middle Eastern countries, such as Iraq, Jordan,
Saudi Arabia, and Lebanon.
 Female predominance is seen in the USA and Britain.10/12/15 4

5. 10/12/15 5

6. 10/12/15 6

7. Diagnosis
The diagnosis of the disease relies on clinical criteria
Initial criteria for diagnosis require the occurrence of at least three
episodes of oral herpetiform or aphthous ulcerations within a 12-
month period
 To confirm the diagnosis, at least two of the following must also be
demonstrated:
1. Recurrent painful genital ulcers that heal with scarring
2. Ophthalmic lesions, including anterior or posterior uveitis, hypopyon,
or retinal vasculitis
3. Skin lesions, including erythema nodosum–like lesions,
pseudofolliculitis, or papulopustular or acneiform lesions
4. Positive results from pathergy skin testing
10/12/15 7

8. Diagnostic criteria
Curth, 1946
 Mason & barnes, 1969
 O Duffy: 1974
 Dilsen: 1986
 Japan: 1972, 1988
 ISG: 1990
 Iran: 1993
 International criteria (ITR-ICBD) (2006)
 ICBD (2010)
10/12/15 8

9. International Study Group criteria for the diagnosis of
Behçet's disease.
`Criterion
Recurrent oral ulceration
Plus any two of the following
Recurrent genital ulceration
Eye lesions
Skin lesions
Positive pathergy test
Required features
Minor aphthous, major aphthous or herpetiform
ulceration observed by physician or patient,
which have recurred at least three times in a 12-
month period
Aphthous ulceration or scarring, observed by
physician or patient
Anterior uveitis, posterior uveitis, or cells in
vitreous on slit lamp examination; or retinal
vasculitis observed by ophthalmologist
Erythema nodosum observed by physician or
patient, pseudofolliculitis or papulopustular
lesions; or acneiform nodules observed by the
physician
Read by physician at 24–48 hours10/12/15 9

10. Sensitivity: 92% , specificity:96%
10/12/15 10

11. Iran criteria(1993)
Oral aphthae 1
Skin lesions (PF, EN) 1
Pathergy test 1
Genital aphthae 2
Eye lesions 2
>= 3 score indicates Behcet disease
10/12/15 11

12. international criteria (ICBD)
Oral aphthosis 1
Skin lesions (PF, EN) 1
Vascular lesions 1
Pathergy test 1
Genital aphthosis 2
Eye lesions 2
3 or more points » Behcet’s Disease
(sensitivity: 96%, specificity: 88%, accuracy: 93.8%)
10/12/15 12

13. New international criteria (new ICBD)
Oral aphthae 2
Skin lesions (PF, EN) 1
Vascular lesions 1
Genital aphthae 2
Eye lesions 2
CNS lesions 1
Pathergy test 1
>= 4 score: sensitivity: 94%, specificity: 90.5%
(International society for Behcet disease, 8-10 July 2010, London)
10/12/15 13

14. Pathology
Histologically, there is combinations of
perivacsular neutrophilic and lymphocytic
infiltration.

 endothelial cell damage or swelling coupled with
fibrinoid necrosis and thrombotic tendency
Association with HLA B51
10/12/15 14

15. 10/12/15 15

16. Clinical features
1.Orogenital ulcers
 Recurrent oral aphthous
ulcers
occur in 98% of cases
Painful, shallow deep with
central necrotic base
Single or in group
Variable size
Present any where in oral
cavity
Persist for 1-2 wks, subside
without scar
Genital ulcers
occurs in about 80–90% of
patients.
The second most common
c/p.
occur usually on the
scrotum in males and on
the vulva, vagina and
cervix in females.
Heal with scar
10/12/15 16

17. 2. Skin involvement
Papulopustular lesions .
Erythema Nodosum.
Vasculitis.
 Sweets syndrome-like lesions.
Pathergy test.
10/12/15 17

18. 3. Ocular Involvement
Ocular involvement in BD is frequent (28.9–80%)
More frequent in males than females.
Anterior, posterior, or panuveitis.
 Retinal vasculitis.
Anterior uveitis with hypopyon.
Iridocyclitis
 Keratitis
 Scleritis,
 Optic neuritis and chorioretinal scars
10/12/15 18

19. 4. Articular Involvement
Occur in 30–70% of BD patients.
 It affects commonly knees, ankles, wrists, and
elbows.
Non deforming arthritis or arthralgia.
10/12/15 19

20. 5.Vascular involvement
Venous system involvement is
more common .
superficial phlebitis.
DVT.
large vein thrombosis
SVC, IVC.
 supra-hepatic, portal vein
thrombosis
 cerebral venous and retina
thrombosis.
Arterial involvement is
less frequent.
Aneurism.
Thrombosis.
Pulmonary arterial
aneurysm.
10/12/15 20

21. 6. Neurological Involvement
 Occurs in 5–10% of patients in BD.
Central nervous system is more frequent.
brainstem and basal ganglia are most commonly
affected.
Dural sinus thrombosis; headache and ICP.
 Arterial vasculitis.
 Aseptic meningitis
10/12/15 21

22. 7. Gastrointestinal Involvement
Frequency vary from country to country.
Higher in Japan and Korea (15-45%).
10/12/15 22

23. 10/12/15 23

24. Investigations
•Leukocytosis.
•ESR, CRP.
•HLA B5
•HLA B51
•Pathergy test.
10/12/15 24

25. 10/12/15 25