Behçet’s disease in clinical practice

Abstract

Behçet’s disease is a chronic relapsing multisystemic vasculitis involving vessels of different diameter. The disease is characterized by involvement of skin and mucosa, eyes, gastrointestinal tract, joints, vessels, genitourinary system and neurological symptoms. According to data, male/female ratio is 2-10:1 The etiology of the disease is still unclear. Diagnosis is mainly based on thorough analysis of clinical manifestations. According to the International Study Group for Behçet’s Disease Diagnostic Criteria, combination of recurrent oral aphthosis and at least any two of the following: severe or scarring genital aphthosis, eye lesions (posterior uveitis, retinal vasculitis), skin lesions (nodular erythema, pseudofolliculitis, acne-like lesions) and positive pathergy test is needed to classify the patient as a patient with Behçet’s disease. Several clinical subtypes of Behçet’s disease are distinguished depending on predominant involvement of different organs and systems, including skin and mucosa, joints, eyes, or neurological manifestations. There are no specific laboratory tests for Behçet’s disease. The treatment approaches are variable depending on clinical manifestations. The effect of systemic corticosteroids, colchicine, azathioprine, chlorambucil, cyclophosphamide, sulfasalazine, methotrexate, cyclosporine, infliximab was proven. All the abovementioned requires the good clinical knowledge on Behçet’s disease from practicing doctors of different specialties, particularly - physicians. A case of highly active acute Behçet’s disease with nose, pharynx, eye and oral cavity mucosa, scrotum skin involvement is presented in the article. Questions of differential diagnosis are reviewed, specific treatment program with efficacy analysis is presented.

Keywords

Behçet’s disease / vasculitis / aphthosis

References

[1] Абдулов А.М., Куравлёва Я.В., Абдуллаходжаев К.А. и др. Случай болезни Бехчета у 13-летней девочки // Клин. дерматол. и венерол. - 2008. - №5. - С. 28-31.

[2] Адаскевич В.П., Катина М.А. Болезнь Бехчета: современные аспекты // Сonsil. Med. Дерматология. - 2009. - №2. - С. 42-47.

[3] Алекберова З.С., Голоева Р.Г., Гусева И.А. Демографические аспекты болезни Бехчета // Рус. мед. ж. - 2010. - №11. - С. 740-743.

[4] Алекберова З.С., Голоева Р.Г., Елонаков А.В. Современная терапия болезни Бехчета // Рус. мед. ж. - 2006. - №8. - С. 578-582.

[5] Нугуманова А.М., Самойлов А.Н. Клинический случай болезни Бехчета в практике офтальмолога // Практ. мед. - 2012. - Т. 2, №4. - С. 128-130.

[6] Ревматология. Клинические рекомендации / Под ред. Е.Л. Насонова. - М.: ГЭОТАР-Медиа, 2010. - 720 с.

[7] Barnes C. Treatment of Behcet’s syndrome // Rheumatology. - 2006. - Vol. 45. - P. 245-247.

RIGHTS & PERMISSIONS

Khamitov R.F., Palmova L.Y., Yakupova Z.N., Khasanova E.R.