1. (Occulo-oral-genital syndrome)
Behçet’s syndrome
(Occulo-oral-genital syndrome)

2. Behçet’s syndrome Hulusi Behçet (1937) Triad:
Recurrent oral aphthous ulcers
Genital ulcers
Ocular inflammation

3. Epidemiology Rare, chronic multisystem disease
Mediterranean, Middle East and Japan
Most prevalent in Turkey (400 / )
15-45 years
Males more commonly affected

4. Etiology & Pathogenesis
Unknown
HLA B 51
Both cellular and humoral immune mechanisms
Abnormal reactivity of neutrophils and lymphocytes-ulceration of the mouth and gut
Deposition of circulating immune complexes in small vessels
Vasculitis of small and large arteries and veins

5. Oral Aphthous Ulcers Recurrent painful oral aphthous ulcers ( %90)
At least three times over a 12 month period
Earliest manifestation
Required for the diagnosis
Heal without scarring

6. oral aphthous ulcers

7. Genital aphthous ulceration- (%70)
Less often than oral ulceration
Large, deep ulcers and often heal with scarring
Men scrotum and penis
-associated with epididymitis
Women-vagina and vulva

8. Ocular inflamation Tends to occur early in the course.
Recurrent and persistent ocular inflamation may leads to visual loss.
Both anterior and posterior uveitis

9. Ocular inflamation
Anterior üveitis - red eye, blurred vision,intense photophobia
Hypopyon-pus in the anterior chamber
Greater threat to vision
Posterior uveitis-vasculitis of carotid and retina

10. Ocular inflamation

11. Cutaneous Manifestations (60%-90%)
Erythema nodosum
tends to ulcerate and heal with scarring and hyperpigmentation
Most commonly in women
Pseudofolliculitis and acneiform nodules
Over the neck,face and extremities

12. Pathergy test
The phenomenon of devoloping an aseptic erythematous papule, nodule and pustules at the needle insertion greater than 2 mm diameter

13. Clinical Manifestations: Vascular
Small-vessel vasculitis is common and accounts for much of the pathologic process in Behçet's disease.
Large vessel vascular involvement occurs in approximately one-third of patients with Behcet's disease.
Superficial and deep venous thrombosis are common.

14. Clinical Manifestations: Vascular
This may lead to stenosis or aneurysm formation. Rupture of such aneurysms may be fatal.
Vascular lesions in the lung, including thrombosis, aneurysm, and arteriobronchial fistula, cause recurrent episodes of dyspnea, cough, chest pain, and hemoptysis.

17. Superficial Thrombophlebitis

18. Erythema nodosum

19. Arthritis (%50) Peripheral arthritis Spondylitis
Oligoarthritis or polyarthritis
Knees most commonly affected
Nondeforming,nonerosive
Spondylitis

20. Gastrointestinal Involvement (25%)
Any portion of the gut from the mouth to the anus can be involved
Most commonly ileum and cecum
Pain,anorexia,rectal bleeding,vomiting and diarrea
Mesenteric arteritis- ischemia of the bowel
Rarely Budd-Chiari syndrome

21. Neurologic Manifestations
Neurologic disease occurs in fewer than one-fifth of patients with Behcet's disease, more frequently in men than women.
Classically, meningitis or meningoencephalitis, neurologic deficits such as motor disturbances and brain-stem symptoms, and psychiatric symptoms including personality changes develop more than five years after Behçet's disease is diagnosed.

22. Recurrent aphtous ulceration (3x/year)
Diagnosis;
Recurrent aphtous ulceration (3x/year)
+ two of the following symptoms & signs
Recurrent genital ulcerations
Eye lesions
Skin lesions
pathergy

23. Neurologic involvement
Treatment
Mucocutaneous lesions
Colchicine
Topical and oral cortico-steoids
Eye involvement
corticosteoids
Immuno-suppressive treatment
Major vein thrombosis
Corticosteoids
Anticoagulant?
Neurologic involvement
Steroids and immuno-suppressive treatment
©ACR