Augey Frédéric, Renaudier Philippe, Nicolas Jean-François
Université Claude-Bernard Lyon I, Department of Immunoallergology, Pavillon Dufourt, Centre Hospitalier Lyon Sud, 69495 Pierre-Benite Cedex. France.
Eur J Dermatol. 2006 Nov-Dec;16(6):669-73.
A retrospective epidemiological survey of generalized pustular psoriasis (GPP) was carried out in France in 2005. 121 dermatological wards received a questionnaire concerning the patients treated in 2004. It related to demographic data, morbidity, mortality, failures and the therapeutic practices of each ward. CNAMTS, the main French health insurance, was also questioned about its registry concerning GPP.112 wards (92.5%) answered the questionnaire, totalling 99 cases (sex ratio male/female: 0.77, mean age 52.5 years +/- 18), which were handled by 46 wards. Incidence and prevalence were estimated in 2004 at a minimum of 0.64 and 1.76/million respectively. Incidence deduced from the CNAMTS data in 1998 and 2001 was similar. The treatment habits were the same in the 46 wards, which used acitretin as first line treatment (89%), followed by methotrexate (8%). High potency dermatocorticosteroids (DC) were most often used (87%). Complications and death were noted in 17% and 2% of the cases respectively, recalcitrant GPP in 42%. Immunobiologics were required in 13% of patients. Univariate analysis showed that treatment failure was related to: i) management in a university ward (OR: 2.9, p = 0.03) probably reflecting the management of the more severe cases ii) prescription of high or very high potency DC as first line local therapy (OR: 7.6, p = 0.05) iii) therapies other than retinoids as first line systemic therapy (OR: 5.5, p = 0.04). The systemic treatment is well codified but future studies will have to confirm the usefulness of DC in the management of GPP.
2005年在法国开展了一项泛发性脓疱型银屑病（GPP）的回顾性流行病学调查。121个皮肤科病房收到了一份关于2004年所治疗患者的问卷。问卷涉及人口统计学数据、发病率、死亡率、治疗失败情况以及每个病房的治疗方法。法国主要的医疗保险机构CNAMTS也被问及有关GPP的登记情况。112个病房（92.5%）回复了问卷，共计99例（男女比例为0.77，平均年龄52.5岁±18岁），由46个病房负责处理。2004年估计发病率和患病率分别至少为0.64/百万和1.76/百万。1998年和2001年从CNAMTS数据推断出的发病率相似。46个病房的治疗习惯相同，均将阿维A作为一线治疗药物（89%），其次是甲氨蝶呤（8%）。最常使用的是高效糖皮质激素（DC）（87%）。分别有17%和2%的病例出现并发症和死亡，42%为顽固性GPP。13%的患者需要使用免疫生物制剂。单因素分析表明，治疗失败与以下因素有关：i）在大学病房接受治疗（比值比：2.9，p = 0.03），这可能反映了对更严重病例的治疗；ii）将高效或超强效DC作为一线局部治疗药物（比值比：7.6，p = 0.05）；iii）除维甲酸类药物外的其他药物作为一线全身治疗药物（比值比：5.5，p = 0.04）。全身治疗已得到很好的规范，但未来的研究必须证实DC在GPP治疗中的有效性。
