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Generalized pustular psoriasis: practical recommendations for Spanish primary care and emergency physicians

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posted on 2023-11-29, 15:20 authored by Raquel Rivera-Diaz, Francisco Epelde, Julio Antonio Heras-Hitos, Ana María Martínez Virto, Airam Jenny Dávalos Marin, María Rosa Senán Sanz, José Manuel Carrascosa

Generalized pustular psoriasis (GPP) is a rare chronic inflammatory skin disease that can lead to life-threatening complications and require emergency medical treatment. Recurrent GPP flares are characterized by the sudden onset of widespread erythematous skin rash with sterile pustules, at times associated with fever, chills, general malaise, and other systemic inflammatory manifestations. Systemic complications such as cardiorespiratory failure, infections, and sepsis are potentially life-threatening and can result in an emergency department visit and/or hospitalization. Acute GPP episodes can be difficult to recognize and diagnose. The low incidence of the disease, its relapsing nature, the unpredictability of flare onset, and the lack of standardized diagnostic criteria are major obstacles to achieving rapid recognition and diagnosis in both the emergency department and the hospital setting.

There is scarce evidence supporting the efficacy and safety of treatments commonly used for GPP; consequently, there is an unmet need for therapies that specifically target the condition. Our aim is to present a multidisciplinary approach to GPP to achieve a rapid diagnosis ensuring that the patient receives the most appropriate treatment for their pathology. The main recommendation for primary care and emergency physicians is to contact a dermatologist immediately for advice or to refer the patient when GPP or a flare is suspected.

Generalized pustular psoriasis (GPP) is a rare and serious skin disease that can cause life-threatening complications and require urgent medical treatment. When someone has a flare-up of GPP, their skin suddenly becomes red and covered with pus-filled bumps not caused by infection. They may also experience fever and chills and feel generally unwell. These flares can be very difficult to diagnose and lead to serious complications such as infections and organ failure, which may require a visit to the emergency department and/or admission to hospital. The diagnosis of GPP can be challenging as it is a rare and unpredictable disease with different types of flare-ups, making it difficult to identify in the emergency department and the hospital. This article shows that the best recommendation for primary care and emergency doctors is to improve their knowledge of this rare condition. Primary care and emergency doctors should immediately contact a dermatologist for advice or referral if they suspect that a patient has GPP or a flare-up of the disease. An approach involving doctors from different specialties can help ensure that patients receive the appropriate and timely care they need.

Funding

Boehringer Ingelheim (BI) has supported and funded the publication of this article. The authors did not receive payment related to the development of the article. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. BI was given the opportunity to review the manuscript for medical and scientific accuracy as well as intellectual property considerations.

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