Generalized Pustular Psoriasis Brazilian Experts Survey: Challenges in Disease Management.

Abstract

Introduction: Generalized pustular psoriasis (GPP) is a rare and severe form of psoriasis. Diagnosis involves several steps due to its rarity and the similarity to other pustular skin conditions. There is a lack of standardized guidelines for managing patients. The objective of this survey was to understand how Brazilian dermatologists manage GPP.

Methods: Three dermatologists, authors of this study, compiled a list of 57 Brazilian dermatologists who had treated patients with GPP in the last 5 years. A questionnaire composed of 28 questions about diagnosis, treatment, and follow-up of patients with GPP was sent to all dermatologists listed.

Results: A total of 32 dermatologists answered the survey. Most were female, had more than 15 years of clinical practice, and had treated at least 3 patients in the last 5 years. The diagnosis was based on the presence of pustules, worsening skin lesions, and erythema. More than half of the participants cited inflammatory markers used for screening. Triggering factors for flares included steroid withdrawal, infection, and stress. Most of them reported that their patients experienced at least one flare per year, lasting 2-4 weeks. Pustules are the first sign of resolution and scaling skin could last more than 6 months. Hospitalization was considered common or very common, often lasting more than 1 week. During GPP flares, the most recommended treatments were cyclosporine. For residual disease treatment, retinoids were the most cited. In addition, 63% of dermatologists think that the options to resolve flares are too slow and 66% consider that options do not prevent new flares.

Conclusions: GPP is a challenging disorder. In Brazil, Brazilian patients with GPP often require longer hospitalization when compared with Europe and USA. A local consensus on GPP management is urgently needed to establish the goals and the standard of care for these patients.