Issue: 2016 > May > letter to the editor

Behçet’s disease: ethnicity and associated conditions

V.M. dos Santos
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Dear Editor,
Behçet’s disease presents with recurrent oral ulceration, genital ulcers, ocular inflammation and skin lesions, and arthritis, neurological and gastrointestinal inflammations.1-3 I read with interest the article by Kappen and colleagues about the prevalence and manifestations of Behçet’s disease, and the correlation of severity and morbidity with patient ethnicity.1 They studied 84 patients with Behçet’s disease of Dutch, Turkish, and Moroccan descent. The prevalence was similar to the countries of ancestry: 1, 39 and 71 per 100,000 for Dutch, Moroccan and Turkish descendants, respectively; and the same was observed about morbidity.1 Notwithstanding, both uveitis and pustules occurred with more frequency in the Netherlands.1
I would like to highlight findings of the Brazilian review by Oliveira and colleagues including 60 Behçet’s disease patients with a mean age of 40 (SD, 10.7) years, and with female-male ratio 1.2:1.0. The total population of Dutch, Turkish and Moroccan descendants is not very large in Brazil; moreover, all patients were born in this country and none were related to specific ethnic groups. As the distribution by ethnicity only revealed 55% mestizos, 36.7% whites, and 8.3% blacks,2 the aim is to compare clinical features in Brazilian patients with those cited by Kappen et al.1 The frequency of clinical manifestations was oral ulcers (100%), genital ulcers (93.3%), cutaneous lesions (71.7%), ocular manifestations (63.3%), arthritis (46.7%), neurological involvement (28.3%), pathergy test (22.7%), thrombosis (13.3%) and gastrointestinal involvement (3.3%).2 Worthy of note, arthritis and erythema nodosum occurred more often in women and papulopustular lesions in men, and prognosis of the entire group was favourable.2 Behçet’s disease has been described in association with heart changes, thromboembolism, and colon tumours, and the concomitance of this condition with patent foramen ovale, antiphospholipid antibodies syndrome, and colon polyps was described in a 49-year-old Brazilian woman.3 Thromboembolic phenomena did not occur in this lady, but cerebral thromboembolism was described in a Japanese man with Behçet’s disease and a similar cardiac anomaly.4 Cases of Behçet’s disease are infrequent in Brazil as in the Netherlands, and few studies have evaluated significant numbers of cases with a confirmed diagnosis.2,3 Therefore, less severe cases in patients with only recurrent oral ulcerations may be underdiagnosed by primary care physicians. The author believes that the articles discussed here may enhance the suspicion index about Behçet’s disease and associated disorders, contributing to reducing misdiagnosis and underdiagnosis, mainly in regions where the ethnic groups under high risk of Behçet’s disease are not well characterised.


The author declares no conflict of interest. No funding or financial support was received.


  1. Kappen JH, van Dijk EHC, Baak-Dijkstra M, et al. Behçet’s disease, hospital-based prevalence and manifestations in the Rotterdam area. Neth J Med. 2015;73:471-7. 
  2. Oliveira ACD, Buosi ALP, Dutra LA, de Souza AWS. Behçet disease: clinical features and management in a Brazilian tertiary hospital. J Clin Rheumatol. 2011;17:416-20. 
  3. Santos VM, Carvalho MRM, Silva FHO, Cançado ACV, Guimarães JPF, Zilcem da Costa Arruda Junior ZC. [Behçet’s disease, anticardiolipin antibodies, patent foramen ovale, and Sweet syndrome – an uncommon association]. Brasília Med. 2013;50:261-5. 
  4. Ishida C, Furui E, Sakashita Y, Yamada M. Embolic stroke with a patent foramen ovale and Behçet disease. Intern Med. 2005;44:326-7.