II. Background

  1. Pronunciation: "Buh shettes"
  2. First described by Hippocrates in fifth century B.C.
  3. Turkish Dermatologist Hulusi Behcet described 1937

III. Epidemiology

  1. Endemic regions
    1. Japan
    2. Eastern Mediterranean (Middle East, Greece, Cyprus, Turkey)
  2. Incidence
    1. United States: 1:100,000
    2. Japan: 670:100,000
  3. Ages: 25-50 years
  4. Gender
    1. Males predominate in Japan
    2. Women predominate in United States

IV. Pathophysiology

  1. Autoimmune Vasculitis
  2. HLA-B51/B5 Allele (found in 57% of Behcet Syndrome patients)

V. Symptoms

  1. Morning stiffness
  2. Oral and Genital Ulcers
  3. Skin rashes
  4. Joint Pain
  5. Neurologic changes

VI. Signs

  1. Mucocutaneous ulcers
    1. Aphthous Stomatitis (100% of cases)
    2. Genital Ulcers (70-90% of cases)
  2. Skin
    1. Papulovesicular, Vasculitis
    2. Erythema Nodosum
    3. Erythema Multiforme
  3. Ocular
    1. Iritis
    2. Iridocyclitis
    3. Chorioretinitis
    4. Hypopyon
    5. Papilledema
    6. Optic Nerve atrophy
  4. Thrombophlebitis
  5. Neurologic
    1. Intracranial Hypertension
    2. Cranial Nerve palsy
    3. Meningitis
  6. Polyarthritis

VIII. Labs: General

  1. Antibodies to SIP1 Carboxy-terminal subunit may be positive
  2. Biopsy
    1. Leukocytoclastic Vasculitis diffusely involving the arterys and veins
  3. Pathergy Test (not sensitive, relatively specific)
    1. See Pathergy Test
    2. Pustule or ulceration forms within 24-48 hours in response to minor Skin Trauma (e.g. SQ Injection)
  4. Non-specific findings
    1. Erythrocyte Sedimentation Rate elevated
    2. Cryoglobulin
    3. Hypergammaglobulinemia
  5. Other rheumatologic labs to consider
    1. Antinuclear Antibody (ANA)
    2. Rheumatoid Factor

IX. Diagnosis: Criteria

  1. Recurrent Aphthous Ulcers (more than 3 per year) and
  2. Two or more of the following criteria
    1. Recurrent Genital Ulcers
    2. Uveitis or other eye lesions
    3. Erythema Nodosum or other skin lesions
    4. Pathergy Test positive (see above)
    5. Biopsy demonstrating diffuse arteritis and venulitis

X. Management: First-Line treatment of mucocutaneous lesions

  1. Pegylated Interferon alfa-2a (Pegasys)
    1. Dose: 6 Million units subcutaneously three times weekly for 3 months
    2. Alpsoy (2002) Arch Dermatol 138(4): 467-71 [PubMed]

XI. Management: Other treatments that have been used for systemic manifestations

  1. Aphthous Ulcers
    1. Topical sucralfate
  2. Uveitis
    1. Topical ocular steroid
  3. Systemic agents
    1. Colchicine 0.6 mg bid
    2. Prednisone 1 mg/kg for severe cases
    3. Azathioprine 2-3 mg/kg/day PO qd
    4. Cyclophosphamide 50-100 mg/day qAM
      1. Take with 64 ounces/day fluid
      2. Observe for Hematuria
    5. Methotrexate 7.5 mg/week or lowest effective dose

XII. Course

  1. Spontaneous regression without treatment may occur

XIII. References

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Related Studies

Ontology: Behcet Syndrome (C0004943)

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

  • Sores in the mouth
  • Sores on the sex organs
  • Other skin sores
  • Swelling of parts of the eye
  • Pain, swelling and stiffness of the joints

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
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