Exploring the Clinical Presentation, Course, and Burden of Disease in Generalized Pustular Psoriasis [Podcast]

Abstract

Generalized pustular psoriasis (GPP) is the most severe form of pustular psoriasis and affects large areas of the body. GPP is a rare disease, and has a variable presentation; thus, its diagnosis is challenging. The onset of symptoms is rapid, with the appearance of painful skin erythema, followed by the widespread eruption of sterile pustules. Acute GPP (called a flare) is often accompanied by systemic symptoms, including high fever, pain in skin lesions, malaise, and fatigue. Approximately half of GPP flares require hospitalization, with an average inpatient duration of 10–14 days. GPP prevalence estimates range from approximately 2–124 cases per million persons, with a female predominance. The most common age of onset of GPP is 40–60 years, although cases have been described in younger adults and children. GPP affects every aspect of patients’ lives and has a high physical and psycho-social impact. Recent research on the interleukin-36 pathway associated with GPP led to the development of a GPP-specific treatment, spesolimab, which was approved by the US FDA in September 2022. This podcast explores the clinical presentation, disease course, and burden of disease in GPP, including differential diagnosis and common triggers of an acute flare.

Keywords:

• generalized pustular psoriasis
• interleukin-36 pathway
• clinical presentation
• disease burden
• diagnosis

Acknowledgments

This podcast was sponsored by Boehringer Ingelheim Pharmaceuticals, Inc. (BIPI).

The authors meet criteria for authorship as recommended by the International Committee of Medical Journal Editors (ICMJE). The authors received no direct compensation related to the development of the manuscript. Editorial support was provided by Katie Crosslin, PhD, and Debra Brocksmith, MB ChB, PhD, both of Envision Pharma Group, which was contracted and compensated by BIPI for this service. BIPI was given the opportunity to review the discussion points for medical and scientific accuracy as well as intellectual property considerations. The authors thank Dr. Uwe Wollina for serving as the moderator, and for reviewing the transcript prior to journal submission.

Disclosure

JFM is a consultant and/or investigator for Amgen, AstraZeneca, Boehringer-Ingelheim, Bristol Myers Squibb, AbbVie, Dermavant, Eli Lilly, Incyte, Moonlake, Novartis, Janssen, UCB, Sanofi, Regeneron, Sun Pharma, Biogen, Pfizer, and LEO Pharma. AZA participates in activities such as speaking, advising, consulting, or providing educational programs for the following companies or other entities: AbbVie, Inc., Amgen, Inc., BMS, Dermavant, Eli Lilly and Company, Great Lakes Clinical Trials, Incyte, Janssen Biotech, Inc., (formerly Centocor Biotech, Inc.), Regeneron Pharmaceuticals, Inc., Sanofi-aventis US, LLC, and UCB Pharma (UCB S.A., Belgium).

Additional information

Funding

Medical writing support, which was provided by Envision Pharma Group, was contracted and funded by Boehringer Ingelheim Pharmaceuticals, Inc.