Behçet syndrome - Anogenital in
Behçet disease is relatively uncommon and is more prevalent in Japan and Middle Eastern and Mediterranean countries. In these populations, it affects men more often than women. 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 be recur less frequently but are more prone to scar formation. Potential complications include fistulae.
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
The possibility of sexually transmitted diseases should always be considered in the evaluation of genital lesions in children and, if present, sexual abuse should be suspected:
Aphthous ulcers – Oral ulcers of Behçet disease tend to recur more frequently, are larger, and can occur in multiples.
Reactive nonsexually related acute genital ulceration (RNSRAGU)
Last Updated: 10/03/2018