Behcet’s Disease

By posted on June 19, 2014 8:47PM
Behcet’s Disease

Behcet’s Disease

Chronic recurrent multisystem condition characterized by
relapsing inflammation and occlusive vasculitis
Triad of oral ulcers, genital ulcers, and inflammatory eye
disease

Diagnosis:
Clinical: 
Major criteria
1. Oral aphthous ulcers (round, discrete borders; heal without scarring)
2. Genital ulcers
3. Skin lesions (erythema nodosum)
4. Ocular disease (75%): recurrent, explosive inflammatory
episodes with active episodes lasting 2–4 weeks
Uveitis (posterior more common than anterior); can present
with nongranulomatous anterior uveitis (usually bilateral;
may have transient hypopyon); occasionally, conjunctivitis,
episcleritis, or keratitis can occur; posterior involvement
with recurrent vascular occlusions, retinal hemorrhages,
exudates, CME, vitritis, traction RD, ischemic optic
neuropathy; may develop glaucoma and cataract.

Minor criteria
1. Arthritis (50%)
2. GI lesions
3. Occlusive vascular lesions of major vessels (vena cava)
4. Migratory thrombophlebitis (33%)
5. CNS involvement (25%; neuro-Behçet’s; meningoencephalitis,
involvement of brain stem, spinal cord, peripheral nerves)
6. Pulmonary artery aneurysm (pathognomonic CXR finding)
7. Interstitial lung changes

Behcetine skin test (cutaneous hypersensitivity):
intradermal puncture; positive test = pustule
formation within minutes
HLA-B51

Treatment: systemic steroids, cytotoxic agents
(chlorambucil most effective for retinal vasculitis,
meningoencephalitis), colchicine (prevents recurrences),
cyclosporine (ciclosporin); plasmapheresis

Behcet’s Disease
Behcet’s Disease

Source: 

Ophthalmology Atlas(Photos of cases)

Behcet’s Disease

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