Medical Musing of the Week: Behcet Disease

Overview

  • Disease marked by recurrent eye and genital lesions as well as ocular involvement.  Autoimmune/autoinflammatory disease with a hereditary component.

Etiology/Pathogenesis

  • Perivasculitis and early neutrophil infiltration w/ endothelial swelling
  • Association with HLA-B*51
  • Mediterranean descent/region, middle east and far east

Clinical Manifestations

  • Recurrent oral and genital ulcers
    • Painful with yellow necrotic base
    • Can occur alone or in clusters
    • Subside in 1-2 weeks w/o scarring
  • Integumentary Involvement
    • 80% of patients also have skin lesions
    • Folliculitis, Erythema Nodosum, Vasculitis (infrequent), sweet syndrome and pyoderma gangrenosum
  • Ocular Involvement
    • 50% of patients experience ocular symptoms
    • iritis, uveitis, retinal vain occlusions, optic neuritis
  • MSK
    • 50% have arthritis or arthralgia
    • Knees and ankles most common locations
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                                               Clinical Manifestations of Behcet’s Disease

Diagnosis

  • Clinical
  • Pathergy Test
    • Introducing a needle under the skin resulting in an abnormal healing and development of ulcers and raised erythematous area demonstrating irregular healing
Positive Pathergy Test.  Increase in inflammation and erythema represents impaired healing
Positive Pathergy Test. Increase in inflammation and erythema represents impaired healing

Treatment

  • Methylprednisilone 
    • 1 g administered intravenously for one to five days,  for progressive, severe, organ or life-threatening disease.
  • Colchicine
    • Effective for mucocutaneous involvement
    • Also associated with decrease in joint involvement for men and women and genital lesions and erythema nodosum in women
  • Azathioprine
    • Patients treatment with steroids and AZA demonstrated less vision deficits and blindness
  • Cyclophosphamide
    • 1 to 2.5 mg/kg per day, orally
    • 1 g or 0.75 to 1 g/m2 by monthly infusion IV
    • Effective for neurologic and vasculitis associated with Behcet’s

Complications

  • 30% develop DVT and PE is a rare complications
  • Blindness may ensue from Panuvitis
  • CNS Behcets: IL-6 is persistently raised in cerebrospinal fluid of these patients

 

 

 

 

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