Behcets Disease

Consider more complex uclers (ie. behcet's) when ulcers last > 3wks or heal with scarring
Behçet disease should be considered in patients with persistent oropharyngeal Aphthous ulcers, with or without anogenital ulcers, associated with systemic findings (eye, nervous system)

Behcets Diagnosis:
Behcet's is a chronic, relapsing, inflammatory disease of recurrent oral aphthous ulcers and numerous systemic manifestations
Most manifestations are due to a vasculitis of all sized vessels in both arterial and venous systems
Behcet's disease involves recurrent oral aphthae and systemic findings:genital aphthae, ocular disease, skin lesions, gastrointestinal involvement, neurologic disease, vascular disease, or arthritis
>50% develop a large vessel thrombosis of some sort
Pathergy and dermatographism may be seen
Pathergy is an erythematous papular or pustular response to local skin injury

Diagnosis:
There is no one diagnostic test; path may reveal vasculitis but is not sensitive
The most sensitive and specific diagnostic criteria came out in 1990 and requires the presence of:
*Recurrent oral aphthae (3x/1 year) plus two of the following without other systemic  diseases

* Recurrent genital aphthae
* Eye lesions (uveitis, vasculitis,etc)
* Skin lesions ( erythema nodosum, papulopustular lesions, or acneiform nodules)
* A positive pathergy test

Treatment:
The literature on Behcet's is limited and conflicting
A cochrane review in 2000 showed that most of the traditional treatments were not beneficial ie) colchicine, steroids, cyclophosphamide.
The European League Against Rheumatism released recommendations in 2008 and 2009, which are based on degree and severity of manifestations, but are only partially evidence based.
Minor disease includes oral and genital ulcers, arthritis and other manifestations that affect quality of life but do not threaten organs.
Minor disease manifestations are treated initially with colchicine, and steroids are only used if colchicine fails
Major disease includes renal, neuro, ophthalmic manifestations
Major disease is treated with hi-dose steroids and a second immunosuppressant including infliximab or cyclophosphamide.

Fitzpatrick's Ch 34: diseases of the mouth
Lancet Diagnostic criteria (attached)
Management of Behcet disease: a systematic literature review for the European League Against Hatemi, et al. Ann Rheum Dis. 2009;68(10):1528-34.Rheumatism evidence-based recommendations for the management of Behcet disease.

(Ellen Eaton MD, 3/28/11)