Department of Medicine, Division of Rheumatology
Dermatology | Skin and Connective Tissue Diseases
INTRODUCTION: Generalized pustular psoriasis (GPP) is a rare, severe, and potentially life-threatening systemic and chronic autoinflammatory disease characterized by sterile, neutrophilic pustules. The standard of care for GPP varies by region, with limited information and experience of flares and their treatment. Our aim was to establish current unmet needs in GPP by better understanding the natural history of GPP, examining how dermatologists diagnose GPP and GPP flares, and establishing the range and adequacy of GPP treatment options currently prescribed by dermatologists.
METHODS: Eligible dermatologists (N = 29) completed a 28-question structured survey, covering ten themes, ranging from GPP diagnostic criteria to GPP symptoms and treatment.
RESULTS: All dermatologists stated that pustules were necessary to diagnose a GPP flare. The most frequently reported triggering factors for GPP were steroid withdrawal (64%), infection (58%), and stress (50%). Most dermatologists indicated that available treatment options for GPP flares were adequate "most" (79%) or "all" (14%) of the time. Despite this reported adequacy, 38% of dermatologists reported that it was at least "somewhat common" for a flare to require hospitalization. Furthermore, 72% of dermatologists indicated that treatments were too slow to control flares, and 66% indicated that treatments did not adequately prevent new flares at least "sometimes".
CONCLUSION: This survey suggests that there are key features of GPP flares, and could initiate discussion around forming consensus guidelines for diagnosis and management. While the results suggest that moderately effective therapies may exist, the need for GPP-specific treatments remains.
Dermatology, Generalized pustular psoriasis, Psoriasis, Survey
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DOI of Published Version
Strober B, Kotowsky N, Medeiros R, Mackey RH, Harrold LR, Valdecantos WC, Flack M, Golembesky AK, Lebwohl M. Unmet Medical Needs in the Treatment and Management of Generalized Pustular Psoriasis Flares: Evidence from a Survey of Corrona Registry Dermatologists. Dermatol Ther (Heidelb). 2021 Apr;11(2):529-541. doi: 10.1007/s13555-021-00493-0. Epub 2021 Feb 27. PMID: 33638115; PMCID: PMC8018987. Link to article on publisher's site
Dermatology and therapy
Strober B, Kotowsky N, Medeiros R, Mackey RH, Harrold LR, Valdecantos WC, Flack M, Golembesky AK, Lebwohl M. (2021). Unmet Medical Needs in the Treatment and Management of Generalized Pustular Psoriasis Flares: Evidence from a Survey of Corrona Registry Dermatologists. Open Access Publications by UMass Chan Authors. https://doi.org/10.1007/s13555-021-00493-0. Retrieved from https://escholarship.umassmed.edu/oapubs/4676
Creative Commons License
This work is licensed under a Creative Commons Attribution-Noncommercial 4.0 License