II. Background
- Pronunciation: "Buh shettes"
- First described by Hippocrates in fifth century B.C.
- Turkish Dermatologist Hulusi Behcet described 1937
III. 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
IV. Pathophysiology
- Autoimmune Vasculitis
- HLA-B51/B5 Allele (found in 57% of Behcet Syndrome patients)
V. Symptoms
- Morning stiffness
- Oral and Genital Ulcers
- Skin rashes
- Joint Pain
- Neurologic changes
VI. 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
VII. Differential Diagnosis
VIII. 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
IX. 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
X. 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]
XI. 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
XII. Course
- Spontaneous regression without treatment may occur
XIII. 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]
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Related Studies
Definition (MEDLINEPLUS) |
Behcet's syndrome is a disease that involves inflammation of the blood vessels. It causes problems in many parts of the body. The most common symptoms are
More serious problems can include meningitis, blood clots, inflammation of the digestive system and blindness. Doctors aren't sure what causes Behcet's. It is rare in the United States, but is common in the Middle East and Asia. It mainly affects people in their 20s and 30s. Diagnosing Behcet's can take a long time, because symptoms may come and go, and it may take months or even years to have all of the symptoms. There is no cure. Treatment focuses on reducing pain and preventing serious problems. Most people can control symptoms with treatment. NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases |
Definition (NCI) | A rare chronic inflammatory disorder of unknown etiology. It is characterized by the development of ulcers in the mouth and genital region and uveitis. Other signs and symptoms include arthritis, deep vein thrombosis and superficial thrombophlebitis. |
Definition (CSP) | rare chronic inflammatory disease involving the small blood vessels; of unknown etiology and characterized by mucocutaneous ulceration in the mouth and genital region and uveitis with hypopyon; the neuroocular form may cause blindness and death; synovitis, thrombophlebitis, gastrointestinal ulcerations, retinal vasculitis, and optic atrophy may occur as well. |
Definition (MSH) | Rare chronic inflammatory disease involving the small blood vessels. It is of unknown etiology and characterized by mucocutaneous ulceration in the mouth and genital region and uveitis with hypopyon. The neuro-ocular form may cause blindness and death. SYNOVITIS; THROMBOPHLEBITIS; gastrointestinal ulcerations; RETINAL VASCULITIS; and OPTIC ATROPHY may occur as well. |
Concepts | Disease or Syndrome (T047) |
MSH | D001528 |
ICD9 | 136.1 |
ICD10 | M35.2 |
SnomedCT | 41225007, 310701003, 154424000 |
LNC | LA17787-5 |
English | Behcet's Syndrome, Behcets Syndrome, Triple-Symptom Complex, BEHCET SYNDROME, BD, BEHCET DISEASE, Behcet Syndrome, BEHCET DIS, Behcet's syndrome, NOS, Behcet's syndrome (diagnosis), Behcets disease, Behcet syndrome, Behçet's disease, Behcet Syndrome [Disease/Finding], behcet syndrome, behcet's syndrome, behcet's disease, behcets syndrome, behcet disease, behcet diseases, behcet's, behcets disease, syndrome behcet's, Behçet Disease, Triple Symptom Complex, Adamantiades-Behcet Disease, Old Silk Route Disease, Behcet Triple Symptom Complex, Behçet's syndrome, Behçet's syndrome (disorder), Behcet's disease (disorder), Behcet Disease, Behðcet's disease, triple symptom complex, Behcet's disease, Behcet's syndrome, Behcet's syndrome (disorder), Behçet, Behcet's disease, NOS, Beh?et's syndrome (disorder), Beh?et's syndrome, Adamantiades-Behcet disease |
Dutch | ziekte van Behçet, Ziekte van Behet, syndroom van Behçet, Tripelsymptomencomplex, Oculo-genitaal syndroom, Oculo-urogenitaal syndroom, Ziekte van Behçet |
French | Maladie de Behcet, Syndrome de Behcet, Trisyndrome de Behçet, Maladie de Behçet, Syndrome oculo-muco-cutané de Behçet, Grande aphtose de Touraine, Syndrome d'Adamantiades-Behçet, Syndrome de Behçet |
German | Behcet-Syndrom, Behcet-Krankheit, Behcet Krankheit, Trisymptomkomplex, Behçet-Krankheit, Behçet-Syndrom, Morbus Behçet |
Swedish | Behcets syndrom |
Japanese | ベーチェットビョウ, ベーチェットショウコウグン, Behcet症候群, ベーチェット病, ベーチェット症候群, 三症状複合体, Behcet病 |
Czech | Behcetův syndrom, Behcetova nemoc, Behcetova choroba, Behçetův syndrom, Behçetova choroba |
Finnish | Behçetin oireyhtymä |
Russian | BEKHCHETA SINDROM, TRIADY SIMPTOMOV KOMPLEKS, БЕХЧЕТА СИНДРОМ, ТРИАДЫ СИМПТОМОВ КОМПЛЕКС |
Italian | Malattia di Behcet, Sindrome di Behcet |
Korean | 베제트 병 |
Spanish | enfermedad de Behçet, enfermedad de Behcet, síndrome de Behcet (concepto no activo), síndrome de Behcet (trastorno), síndrome de Behcet, Complejo de Síntoma Triple, Enfermedad de Behçet, Síndrome de Behçet |
Polish | Zespół Behceta, Choroba Behceta, Zespół Hulusi-Behceta |
Hungarian | Behçet syndroma, Behçet-betegség, Behçet-syndroma, Behçet betegség |
Norwegian | Bechets syndrom, Behcets sykdom |
Portuguese | Complexo de Triplo Sintoma, Doença de Behçet, Síndrome de Behçet |