Updated: 2/9/2018

Behcet Syndrome

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Snapshot
  • A 40-year-old East Asian man presents for painful oral and genital ulcers. He reports that he has had these ulcers for about a week. About a year ago, he moved from Malaysia to the United States. He reports that he is currently not sexually active and has had a test for HIV half a year ago, which was negative. He is in a 3-year monogamous relationship. Physical exam shows 3 aphthous ulcers in his oral mucosa. Genital exam reveals multiple erythematous ulcers along his penis. Additionally, there are soft subcutaneous nodules along his bilateral shins. Labs reveal increased inflammatory markers. He is given topical corticosteroids.
Introduction
  • Clinical definition
    • a systemic small and large vessel vasculitis
  • Epidemiology
    • incidence
      • most common in Asia, Mediterranean regions, and the Middle East
    • demographics
      • male > female
      • 20-40 years of age
    • risk factors
      • geographic area
      • family history
  • Pathogenesis
    • thought to be an autoimmune process that is triggered by an environmental or infectious source, especially in a genetically predisposed person
    • mechanism involves increased cytokines, proliferation of T-cells, and hyperactive neutrophils
  • Associated conditions
    • erythema nodosum
Presentation
  • Symptoms
    • arthritis
    • enthesitis
    • can have vascular thrombosis or aneurysms from vasculitis
  • Physical exam
    • recurrent oral aphthous ulcers
    • genital ulcers
    • bilateral ocular lesions
      • slit lamp testing
        • nongranulomatous uveitis
        • anterior uveitis
    • skin lesions
      • papules and pustules
      • acneiform nodules
      • erythema nodosum
      • cutaneous ulcers
      • pathergy is common
        • papules or pustules in areas of previous trauma
    • cranial nerve palsies may occur
Imaging
  • Magnetic resonance imaging (MRI) of head
    • indication
      • used if neurologic symptoms are present
  • Computed tomography (CT) of chest
    • indication
      • used if pulmonary involvement (pulmonary artery occlusion or aneurysm) is suspected
Studies
  • Labs
    • ↑ inflammatory markers
      • erythrocyte sedimentation rate
      • C-reactive protein
      • leukocytes
    • autoantibodies typically absent
  • Biopsy
    • indications
      • used to confirm diagnosis
      • can differentiate Behcet syndrome from Crohn disease
    • findings
      • leukocytoclastic vasculitis or lymphocytic vasculitis
      • thrombosis
  • Making the diagnosis
    • most cases are clinically diagnosed
Differential
  • Herpes genitalis
  • Syphilis
  • Inflammatory bowel disease
Treatment
  • Management approach
    • based on affected organs and discussion of side effects with patients
  • Medical
    • topical corticosteroids
      • indication
        • isolated oral or genital ulcers
    • colchicine
      • indications
        • erythema nodosum
        • arthritis
    • other systemic immunosuppression
      • indications
        • other organ involvement, including ocular, gastrointestinal, and neurologic
        • drugs
          • corticosteroids
          • cyclosporine
          • infliximab
          • cyclophosphamide
          • azathioprine
Complications
  • Blindness from ocular disease
  • Thrombosis 
 

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Questions (1)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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