Behçet Disease



Behçet Disease


Brian J. Hall, MD

Bruce R. Smoller, MD

David J. DiCaudo, MD










These are indurated plaques on the arm with superficial erosions in a patient with genital and oral ulcerations. Biopsy showed LCV and subcorneal neutrophilic pustulosis compatible with Behçet disease.






Behçet disease involving the scrotum. Deep dermal vessels image are the focus of a dense inflammatory infiltrate associated with vasculitis.


TERMINOLOGY


Abbreviations



  • Behçet disease (BD)


Synonyms



  • Behçet syndrome, oculo-oral-genital syndrome, aphthosis


Definitions



  • Systemic illness defined by the presence of recurrent oral aphthous ulcerations plus any 2 of the following



    • Genital ulcerations, skin lesions (erythema nodosum [EN]-like lesions, pseudofolliculitis, papulopustular lesions, or postadolescent acneiform lesions), eye lesions (retinal vasculitis, anterior or posterior uveitis), or positive pathergy test


    • Oral aphthous ulcers must recur at least 3x over 12-month period


CLINICAL ISSUES


Epidemiology



  • Age



    • Young adults


    • Peak incidence in 3rd decade


  • Gender



    • Male predominance


  • Ethnicity



    • Worldwide distribution but highest incidence in Pacific rim and Mediterranean countries

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Behçet Disease
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