Generalized pustular psoriasis (Zumbusch): a French epidemiological survey

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.