Behçet syndrome - Anogenital in
Behçet disease is relatively uncommon and is more prevalent in Japan and Middle Eastern and Mediterranean countries. While Behçet disease has an equal sex distribution overall, there is male predominance in Middle Eastern and Mediterranean patients and female predominance in Japan and Korea. The onset of symptoms typically occurs during the third or fourth decades of life. Rarely, it can present during childhood, and usually in children with a positive family history of the syndrome. Behçet disease is strongly associated with the HLA-B51 allele, which is present in more than 80% of Asian patients with Behçet disease.
Clinically, Behçet disease is characterized by recurrent oropharyngeal and genital ulcers and ocular involvement. Oral ulcers are the initial presenting sign in up to 80% of patients and can lead to scarring, dysphagia, and odynophagia. Genital ulcers present similarly to oropharyngeal lesions; they tend to recur less frequently but are more prone to scar formation. Potential complications include epididymo-orchitis.
Ocular disease, seen more often in men, is characterized by episodes of bilateral, nongranulomatous anterior and/or posterior uveitis. It usually appears 2-3 years after the onset of oral and/or genital ulcers, although it can be the initial manifestation in up to 20% of cases. Approximately 1 in 4 patients with ocular disease develop blindness.
Other cutaneous manifestations include erythema nodosum-like lesions, folliculitis-like lesions, erythema multiforme-like lesions, Sweet syndrome-like lesions, subcutaneous thrombophlebitis, and palpable purpura.
Related to Behçet syndrome is Hughes-Stovin syndrome, in which patients don't have the clinical criteria for Behçet syndrome but have a vasculitis and may have pulmonary aneurysm.
M35.2 – Behçet's disease
310701003 – Behcet's syndrome
Differential Diagnosis & Pitfalls
- Primary syphilis
- Granuloma inguinale
- Aphthous ulcers – oral ulcers of Behçet disease tend to recur more frequently, are larger, and can occur in multiples
- Crohn disease
- Ulcerative colitis
- Pyoderma gangrenosum
- Calciphylaxis – rarely may present with a painful ulcer on the penis
- Balanitis xerotica obliterans