2. Behçet's disease (BEH-chets)
• Also called Behçet's syndrome, Morbus
Behçet, or Silk Road disease, is a rare
immune-mediated systemic vasculitis.
• 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
3. Behçet's disease (BEH-chets)
• Also called Behçet's syndrome, Morbus
Behçet, or Silk Road disease, is a rare
immune-mediated systemic vasculitis.
• 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
4. Behçet's disease (BEH-chets)
• Also called Behçet's syndrome, Morbus
Behçet, or Silk Road disease, is a rare
immune-mediated systemic vasculitis.
• 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
5. Behcet’s Syndrome
Definition:
Behçet's syndrome is a multisystem disorder
presenting with-
• Recurrent oral and genital ulcerations and
• Ocular involvement
• Diagnosis is clinical, based on internationally
agreed diagnostic criteria
6. Behcet’s Syndrome
Incidence, and Prevalence
• Affects young males and females from the
Mediterranean region, Middle East, and Far
East, suggesting a link with the ancient Silk
Route.
• Males and females affected equally
• Males often have more severe disease
• Blacks are rarely affected
7. Behcet’s Syndrome
Diagnostic Criteria of Behçet's Disease
• Recurrent oral ulceration plus two of the
following:
–Recurrent genital ulceration
–Eye lesions
–Skin lesions
–Pathergy test
8. Behcet’s Syndrome: Pathogenesis
Etiology and pathogenesis - obscure
Main lesions:
• Systemic perivasculitis, early neutrophil
infiltration
• Endothelial swelling & panvasculitis
• Vasculitis of vasa vasorum -formation of
pseudoaneurysms
9. Behcet’s Syndrome: Pathogenesis
Etiology and pathogenesis - obscure
Main lesion:
• Panvasculitis
Also seen:
• Increased numbers of infiltrating CD4+ T cells
• Circulating autoantibodies against –
–Enolase of endothelial cells
–Selenium binding protein
–Anti-saccharomyces cerevisiae antibodies (also
in Crohn's)
• Association with HLA-B*51
10. Behcet’s- Clinical Features:
1. Orogenital ulceration
• Recurrent aphthous
ulcers are a sine qua non
• Painful, shallow /deep
with central necrotic
base
• Singly or in crops
• Variable size
• Located anywhere in the
oral cavity
• Persist X 1–2 weeks,
subside without scars
• The genital ulcers –
• less common, but more
specific
• Painful
• Spare the glans penis and
urethra, and
• produce scrotal scars
11. Behcet’s- Clinical Features:
2. Skin involvement -80%
• Folliculitis
• Erythema nodosum
• An acne-like exanthem
• Vasculitis (infrequent)
• Sweet's syndrome
• Pyoderma gangrenosum
• Nonspecific skin inflammatory reactivity to any
scratches or intradermal saline injection
(pathergy test) is a common and specific
manifestation.
12. Behcet’s- Clinical Features:
3. Eye involvement in 50%
• Scarring and bilateral pan-uveitis
• Iritis, posterior uveitis,
• Retinal vessel occlusions, and
• Optic neuritis
19. Treatment: Behcet's Syndrome
• The severity of the syndrome usually abates
with time.
Apart from patients with
• CNS-Behçet's syndrome and
• Major vessel disease, the life expectancy
seems to be normal
• The only other serious complication is
blindness.
