Behçet Disease
Brian J. Hall, MD
Bruce R. Smoller, MD
David J. DiCaudo, MD
Key Facts
Microscopic Pathology
Diagnosis is primarily clinical
Oral and genital lesions often show nonspecific ulceration ± associated LCV or lymphocytic vasculitis
Top Differential Diagnoses
Very broad due to variable and nonspecific histologic features (clinical history very important)
Includes neutrophilic dermatoses, connective tissue disorders, EN, vasculitis, folliculitis, infection, and others
Diagnostic Checklist
No histologic findings are pathognomonic; clinicopathologic correlation is crucial in arriving at correct diagnosis
Main role of biopsy: Confirm clinical suspicion of disease
 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  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
Presentation
Very painful, recurrent ulcerations invariably present
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