| name: | Behcet's Syndrome |
| also known as: | Behcet Syndrome; Bechet's Syndrome; Franceshetti-Valero Syndrome; ICD 136.1 |
| also see: | Autoimmune Disease; Connective Tissue Disease; Reiter's Syndrome; Erythema Multiforme; Systemic Lupus; Crohn's Disease; Sweet's Syndrome; Amyloidosis; ICD |
| description: | Behcet's is an illness characterized by recurrent episodes of inflammation involving multiple systems, including the skin, mucous membranes, eyes, genitals, joints, brain, nerves, intestinal tract, kidneys, and blood vessels. Characterized by mucocutaneous ulcerations, skin rash, arthritis, colitis, uveitis, thrombophlebititis. Cause is unknown but a viral etiology and an autoimmune component are suspected. Other suspected but unproven causes include heavy metal exposure, pesticides, English walnuts, Ginkgo nuts. The disease occurs in adults, more common in men than women, and seems to exist along the ancient silk route from Asia to the Mediterranean. It is uncommon is the USA but should be suspected in an adult with genital and oral ulcers in association with unexplained eye problems. Male > female. |
| signs & symptoms: | Includes recurrent, painful oral ulcers, penis and scrotal ulcers, vagina and vulva ulcers, as well as eye inflammation including uveitis, keratitis, optic neuritis, skin papules including erythema nodosum and superficial thrombophlebitis, joint pain and swelling, morning stiffness, superficial thrombophlebitis, an inflammatory meningitis, and an intestinal inflammation similar to Crohn's disease. |
| diagnosis: | Based on signs, symptoms, history and exam. No direct test, diagnosis is clinical. HLA-B5 antigen often present. Diagnosis based on clinical presentation rather than a diagnostic test. Often immune profiles such as ANA profile are used to follow the course of th disease. Differential diagnosis includes: systemic lupus, HIV infection, endocarditis, aphthous oral ulcer, erythema multiforme, herpes simplex, herpes simplex keratitis, Reiter's syndrome, syphilis, inflammatory bowel disease arthritis, Stevens-Johnson syndrome. |
| treatment: | Symptomatic treatment might include colchicine, a gout medication for erythema multiforme and joint pain, topical steroids, oral prednisone steroids such as prednisone, and on occasion, immunosuppressive drugs such as imuran, cyclosporine, or methotrexate. Resistant cases might improve with tacrolimus, thalidomide, Interferon alpha, cyclophosphamide, levamisole. |
| prevention: | None. |
| outcome: | The disease is chronic, usually not curable, but is manageable. Can rarely progress to paralysis, blindness, amyloidosis, aneurysm, hypercoagulable state, death. |
skynetMD suggests the following:
| if: | If the person develops symptoms suggestive of Behcet's syndrome, especially oral ulcer, genital ulcers, and eye inflammation |
| go to: | Go to the doctor. |
Last updated 6/4/2010