Behçet’s Disease

Multisystem disease with symptomatic variations
 
Mechanism
  • Vaculite sistémica
  • Dermatose neutrofílica
  • Síndrome autoinflamatório
 
Apresentação clínica
  • Skin/mucous membranes
    • Aphthous stomatitis (70%)
    • Genital ulcerations (scrotum, penis, vulva)
    • Acrofacial vesiculopustules
    • Panniculitic erythema nodosum-like lesions
    • Pathergy
  • Systemic
    • Eye findings (90%), most commonly posterior uveitis
    • Non-erosive mono or polyarthritis (50%), small joints
    • GI disease (if diagnosed IBD, then Behçet’s “excluded”)
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Epidemiology: seen mostly in japanese, middle east and mediterranean
  • HLA-B51 association
  • “Silk road”
    • The Silk Road was a network of trade routes that connected the East and West, stretching from the Mediterranean to China. It was used as a trade route for goods such as silk, spices, precious metals, and other luxury items, as well as for the exchange of ideas and cultural practices. The Silk Road played a significant role in cultural exchange and economic development between the regions it connected.
    • Behçet is most commonly found in the countries along the Silk Road, such as Turkey, Iran, and Japan
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Critérios de diagnóstico
  • International Study Group
  • Major
    • Aphthous oral ulceration occuring at least 3 times in 12 months
  • Minor (need 2)
    • Recurrent genital ulceration
    • Eye lesions: anterior or posterior uveitis, cells in vitreous humps, or retinal vasculitis
    • Skin lesions: EN, papulopustules, acneiform lesions
    • Pathergy test at 24-48h
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Histologia
  • Neutrophilic angiocentric infiltrate with leukocytoclastic (early) or lymphocytic (late) vasculitis
  • Usually inconclusive
  • Mixed findings of vascular injury, neutrophils, lymphocytes, lack of antibodies, and sometimes vasculitis makes categorization difficult
  • Rule out HSV
 
Treatment
  • Aggressive ophthalmology therapy
  • Nutritional considerations (because of oral ulcers and possible GI involvement)
  • Topical anesthetics
  • Same as pyoderma gangrenosum
  • Apremilast recently FDA approved
    • Inhibits phosphodiesterase 4 (PDE4), which reduces inflammation in the body
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