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Behçet’s disease may occur in ethnic groups that were previously thought to have a negligible risk of it, which raises the possibility that environmental factors contribute to the disease. Consideration needs to be given to this in the differential diagnosis of severe intraocular inflammation, particularly in the presence of mucosal aphthosis.
Behçet’s disease is thought to occur mainly in countries bordering the Mediterranean, in Asia, and in the Far East. A case series of eight patients of west African or Afro-Caribbean origin in London showed, however, that seven patients satisfied the criteria of the International Study Group for Behçet’s disease, and in the eighth patient, who had ocular and neurological involvement, typical histological changes were seen at necropsy.
The genetic association of the MHC allele HLA-B51 may define risk of the disease, particularly for ocular involvement. All patients had resided in the United Kingdom for several years, and six of them were B51 negative.
The rarity of the disease in populations in central and west Africa may be due to the fact that the HLA-B51 allele is not often found in this region. Another possible explanation may be a bias in case ascertainment, which depends on recognition of the disease and access to medical care.