[摩洛哥白塞病患者诊断标准的比较]

El Fajri S, Benchikhi H, Jarmouni R, Lakhdar H

Service de Dermatologie-Vénérologie, CHU Ibn Rochd, quartier des hôpitaux, Casablanca, Maroc.

Ann Dermatol Venereol. 2000 Dec;127(12):1068-72.

BACKGROUND

Behçet's disease is a vasculitis with diverse systemic manifestations usually involving the skin, eyes, joints and nerves. Clinical diagnosis is based on several signs, generally using the criteria established by the International Group for the Study of Behçet's disease, by O'Duffy, by Hamza, by Mason and Barnes and by the Japanese Committee. The purpose of this retrospective study was to assess the frequency of clinical manifestations in Behçet's disease and to apply the five diagnostic criteria scorings cited above to our patients to determine their sensitivity.

PATIENTS AND METHODS

Between January 1988 and December 1997, 110 cases of Behçet's disease were seen at our Dermatology Unit in Casablanca as inpatients or outpatients. Complete history and clinical data including results of the ophthalmology exam and skin tests were available for 70 patients (29 men and 41 women, mean age 31 years).

RESULTS

All 70 patients had buccal aphtosis and 88 p. 100 had genital aphtosis. Ocular signs were found in 36 p. 100 of the patients and the skin test was positive in 57 p. 100. According to the criteria of the International Group for the Study of Behçet's disease, 65 patients (93 p. 100) had Behçet's disease. For the Japanese Committee criteria there were 63 (90 p. 100), for the O'Duffy criteria 48 (68 p. 100), for Hamza criteria 45 (64 p. 100) and for Mason and Barnes criteria 35 (50 p. 100).

DISCUSSION

Our series is comparable to other Mediterranean series observed in dermatology units, but our patients had fewer systemic manifestations compared with another Moroccan series reported from an internal medicine ward. This could be due to recruitment bias and different medical education. For positive diagnosis of Behçet's disease, the criteria established by the International Group for the Study of Behçet's disease, the Japanese Committee, O'Duffy and Hamza enabled the diagnosis in the majority of the cases. Fifty percent of the patients had all the criteria described in these scores. These criteria would appear to be both overly descriptive, with the risk of confounding other disease states such as enterocolopathy, or underly descriptive, making it impossible to classify certain patients as having Behçet's disease.

背景

白塞病是一种血管炎，有多种全身表现，通常累及皮肤、眼睛、关节和神经。临床诊断基于多种体征，一般采用白塞病国际研究小组、奥达菲、哈姆扎、梅森和巴恩斯以及日本委员会制定的标准。这项回顾性研究的目的是评估白塞病临床表现的发生率，并将上述五种诊断标准评分应用于我们的患者，以确定其敏感性。

患者与方法

1988年1月至1997年12月期间，我们在卡萨布兰卡皮肤科病房诊治了110例白塞病患者，包括住院患者和门诊患者。70例患者（29名男性和41名女性，平均年龄31岁）有完整的病史和临床资料，包括眼科检查和皮肤试验结果。

结果

所有70例患者均有口腔溃疡，88%有生殖器溃疡。36%的患者有眼部体征，57%的患者皮肤试验呈阳性。根据白塞病国际研究小组的标准，65例患者（93%）患有白塞病。按照日本委员会标准有63例（90%），按照奥达菲标准有48例（68%），按照哈姆扎标准有45例（64%），按照梅森和巴恩斯标准有35例（50%）。

讨论

我们的系列病例与皮肤科病房观察到的其他地中海地区系列病例具有可比性，但与内科病房报告的另一个摩洛哥系列病例相比，我们的患者全身表现较少。这可能是由于招募偏倚和不同的医学教育。对于白塞病的阳性诊断，白塞病国际研究小组、日本委员会、奥达菲和哈姆扎制定的标准在大多数病例中能够作出诊断。50%的患者符合这些评分中描述的所有标准。这些标准似乎要么描述过于详细，有混淆其他疾病状态（如小肠结肠炎）的风险，要么描述不够详细，使得无法将某些患者归类为患有白塞病。