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