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Generalized Pustular Psoriasis (GPP) Overview

GPP is a chronic, neutrophilic inflammatory skin disease associated with a considerable clinical burden.1-3 It is estimated to affect 1 in 10,000 people in the US4 with a higher predominance in females.5,6 It is a heterogenous disease with symptoms presenting on a spectrum1,2 that includes:

  • Chronic skin lesions with underlying inflammation7

  • Sudden onset of rapidly disseminating erythema covered with sterile pustules, crusts and scales, often associated with systemic symptoms (fever, pain, fatigue, malaise)2

GPP is recognized as a separate clinical entity from other forms of psoriasis (ICD-10 code: L40.1); IL-36 pathway is the key driver of GPP and elicits response to treatment, while the IL-17/IL-23 pathway drives plaque psoriasis.8,9

It can be relapsing or persistent, which may require chronic management.1,2 GPP can greatly affect patients’ QOL.10

A National Psoriasis Foundation 2024 consensus statement advocates for timely access to FDA-approved therapies for GPP because delays can increase the risk of mortality in patients with this rapidly progressing disease.11

GPP Disease Severity Over Time

References

  1. Navarini AA, et al. J Eur Acad Dermatol Venereol. 2017;31(11):1792-1799.

  2. Puig L, et al. J Acad Dermatol Venereol. 2023;37(4):737-752. 

  3. Kharawala S, et al. Exp Rev Clin Immunol. 2020;16:239-252. 

  4. Kam O, et al. J Eur Acad Dermatol Venereol. 2024;38(6):e536-e537.

  5. Crowley J, et al. Journal of Psoriasis and Psoriatic Arthritis. 2021;6(3):151-158.

  6. Jin H, et al. J Dermatol. 2015;42(7):674-678.

  7. Onoufriadis A, et al. Amer J Human Genetics. 2011;89:432-437.

  8. Marrakchi S, Puig L. Am J Clin Dermatol. 2022;23(Suppl 1):13-9.

  9. Zema CL, et al. JAMA Dermatol. 2022;158(10):1142-8.

  10. Reisner DV, et al. Am J Clin Dermatol. 2022;23(Suppl 1):65-71.

  11. Armstrong AW, et al. J Am Acad Dermatol. 2024;90:727-30.

  12. Strober B, et al. AAD 2021. Poster 26588.

  13. Ly K, et al. Psoriasis (Auckl). 2019;9:37-42.

  14. Morita A, et al. Lancet. 2023;402(10412):1541-1551.

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