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Behcet's Syndrome
Aka: Behcet's Syndrome, Behcet's Disease, Behcet Syndrome, Adamantiades-Behcet Syndrome, Behðcet's Disease, Behcet Triple Sydrome Complex- See Also
- Background
- Pronunciation: "Buh shettes"
- First described by Hippocrates in fifth century B.C.
- Turkish Dermatologist Hulusi Behcet described 1937
- Epidemiology
- Endemic regions
- Japan
- Eastern Mediterranean (Middle East, Greece, Cyprus, Turkey)
- Incidence
- United States: 1:100,000
- Japan: 670:100,000
- Ages: 25-50 years
- Gender
- Males predominate in Japan
- Women predominate in United States
- Endemic regions
- Pathophysiology
- Autoimmune Vasculitis
- HLA-B51/B5 Allele (found in 57% of Behcet Syndrome patients)
- Symptoms
- Morning stiffness
- Oral and Genital Ulcers
- Skin rashes
- Joint Pain
- Neurologic changes
- Signs
- Mucocutaneous ulcers
- Aphthous Stomatitis (100% of cases)
- Genital Ulcers (70-90% of cases)
- Skin
- Papulovesicular, Vasculitis
- Erythema Nodosum
- Erythema Multiforme
- Ocular
- Iritis
- Iridocyclitis
- Chorioretinitis
- Hypopyon
- Papilledema
- Optic Nerve atrophy
- Thrombophlebitis
- Neurologic
- Intracranial Hypertension
- Cranial Nerve palsy
- Meningitis
- Polyarthritis
- Mucocutaneous ulcers
- Differential Diagnosis
- Labs: General
- Antibodies to SIP1 Carboxy-terminal subunit may be positive
- Biopsy
- Leukocytoclastic Vasculitis diffusely involving the arterys and veins
- Pathergy Test (not sensitive, relatively specific)
- See Pathergy Test
- Pustule or ulceration forms within 24-48 hours in response to minor Skin Trauma (e.g. SQ Injection)
- Non-specific findings
- Erythrocyte Sedimentation Rate elevated
- Cryoglobulin
- Hypergammaglobulinemia
- Other rheumatologic labs to consider
- Diagnosis: Criteria
- Recurrent Aphthous Ulcers (more than 3 per year) and
- Two or more of the following criteria
- Recurrent Genital Ulcers
- Uveitis or other eye lesions
- Erythema Nodosum or other skin lesions
- Pathergy Test positive (see above)
- Biopsy demonstrating diffuse arteritis and venulitis
- Management: First-Line treatment of mucocutaneous lesions
- Pegylated Interferon alfa-2a (Pegasys)
- Dose: 6 Million units subcutaneously three times weekly for 3 months
- Alpsoy (2002) Arch Dermatol 138(4): 467-71 [PubMed]
- Pegylated Interferon alfa-2a (Pegasys)
- Management: Other treatments that have been used for systemic manifestations
- Aphthous Ulcers
- Topical sucralfate
- Uveitis
- Topical ocular steroid
- Systemic agents
- Colchicine 0.6 mg bid
- Prednisone 1 mg/kg for severe cases
- Azathioprine 2-3 mg/kg/day PO qd
- Cyclophosphamide 50-100 mg/day qAM
- Take with 64 ounces/day fluid
- Observe for Hematuria
- Methotrexate 7.5 mg/week or lowest effective dose
- Aphthous Ulcers
- Course
- Spontaneous regression without treatment may occur
- References
- Ghate in Ruddy (2001) Kelley's Rheumatology, p. 1205-8
- Ghate (1999) J Am Acad Dermatol 40(1):1-18 [PubMed]
- Roett (2012) Am Fam Physician 85(3): 254-62 [PubMed]