Exploring the Clinical Features, Immunopathogenesis and Approach to Diagnosis for Generalized Pustular Psoriasis [Podcast]

Abstract

Generalized pustular psoriasis is a rare presentation of psoriatic disease and is characterized by the acute onset of diffuse superficial pustules on the skin. These pustules can often coalesce, forming what’s known as ‘lakes of pus’ that are most often seen on the trunk and on skin folds. GPP flares are often accompanied by systemic symptoms, including fever, malaise, and edema. The interleukin (IL)-36 pathway plays a central role in the development of GPP, although several other genes may be associated. The rarity of GPP makes its diagnosis challenging and it could be mistaken for an infectious condition or other types of pustular psoriasis, including unstable forms of plaque psoriasis that may present with pustules. Performing a thorough skin examination and obtaining a detailed history are vital to exclude these differential diagnoses. Incorrect or late diagnosis, inadequate or delayed treatment, and lack of specialist referrals may contribute to increased disease severity and can have a debilitating impact on patients’ quality of life. In this podcast, two US-based dermatologists discuss the clinical characteristics of GPP, highlight the central role of IL-36 in immunopathogenesis, and share practical approaches to recognizing and diagnosing the disease.

Keywords:

• Diagnosis
• generalized pustular psoriasis
• genetics
• immunopathogenesis
• interleukin-36 pathway
• quality of life

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 Allison Craig, PharmD, both of Elevate Scientific Solutions, which was contracted and compensated by Boehringer Ingelheim Pharmaceuticals, Inc. (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

TB is currently aprincipal investigator for studies being sponsored by AbbVie, Castle, CorEvitas, Dermavant, Galderma, Mindera, and Pfizer. She has additional research funding from Novartis and Regeneron. She has served as an advisor for AbbVie, Arcutis, Boehringer-Ingelheim, Bristol Myers Squibb, Janssen, Leo, Eli Lilly and Company, Pfizer, Novartis, Sun, and UCB.

JEH has served as a consultant and/or advisor for AbbVie, Arcutis, Boehringer Ingelheim, BMS, Janssen, LearnSkin, LEO, Lilly, Novartis, Pfizer, Regeneron-Sanofi Genzyme, and UCB. He also currently serves on the medical board and scientific advisory committee of the National Psoriasis Foundation and is a councilor in the International Psoriasis Council.

Additional information

Funding

Medical writing support, which was provided by Elevate Scientific Solutions, was contracted and funded by Boehringer Ingelheim Pharmaceuticals, Inc. (BIPI).