BEHÇET’S DISEASE

Behçet’s disease (BD) which is classified among vasculitides is a systemic disease with various manifestations. Its clinical course is characterized by attacks and remissions. Till now, two nationwide surveys of BD from Iran and Japan and 4 major case series from Turkey, Korea, Morocco and England have been reported. Clinical picture of BD is dominated by mucous membrane manifestations, including oral aphthosis - seen in 96.8% of patients in Iran, 98.2% in Japan, 100% in Turkey, 97.5% in Korea, 100% in Morocco and 100% in England- and genital aphthosis which is seen less frequently- 65.3% in Iran, 73.2% in Japan, 88.2% in Turkey, 56.7% in Korea, 83.5% in Morocco and 89% in England. Skin aphthosis is not frequent but it is the most characteristic lesion of BD. Ocular manifestations include anterior uveitis, posterior uveitis and retinal vasculitis. Joint manifestations include arthralgia, monoarthritis, oligo/poly arthritis, and ankylosing spondylitis. Other manifestations include neurological, gastrointestinal and cardiopulmonary manifestations, vascular involvement, orchitis and epididymitis. Erythrocyte sedimentation rate is usually elevated. Urinary abnormalities are infrequent and transient. Positive pathergy test has been reported in 57.4% of patients in Iran, 44% in Japan, 57% in Turkey, 40% in Korea, 68% in Morocco and 32% in England. Lesions usually heal without sequela, except for lesions of eyes, brain and vascular system. The major cause of morbidity is the ocular lesion, which could lead to severe loss of vision or blindness

Dermatoscopic and mucoscopic features of lesions in patients with Behcet's disease

Objective: Behcet's disease (BD), as a vasculitis, can affect small and large vessels. As dermatoscopy has been shown to improve the accuracy in diagnosis of various skin lesions especially vascular patterns, we set this study to find if there is any characteristic pattern in the dermatoscopy of Behcet's mucocutaneous lesions.Methods: This prospective cross-sectional observational study designed to evaluate dermatoscopic features of Behcet's mucocutaneous lesions. Fifty six consecutive patients presenting at the outpatient clinic of the BD Research Unit were included. If present, for each patient one oral, one skin and one genital lesion were evaluated by dermatoscopy. When indicated, pathergy test was performed according to a standard protocol and the results were evaluated by dermatoscopy.Results: A total of 40 oral, 8 genital, 14 skin lesions and 14 pathergy tests were evaluated by dermatoscopy. While vascular component was the most prominent feature in oral aphthae, this component was less prominent in genital lesions. Dot vessels were the most common form of vessels in both oral and skin lesions. All the oral lesions were characterized by a central white structureless area. Skin lesions were characterized by a red structureless background. In pathergy tests, negative pricks showed absence of specific features while positive pricks were characterized by a structureless background in pink, purple or red. No obvious vascular component was detected in any of the pricks.Conclusion: It seems that these findings have no specific clues for the diagnosis of BD, but our study is the first study in this field and the findings may give way to further investigations

The International Criteria for Behçet's Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria

Behçet's disease (BD) is a chronic, relapsing, inflammatory vascular disease with no pathognomonic test. Low sensitivity of the currently applied International Study Group (ISG) clinical diagnostic criteria led to their reassessment