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UNDERSTANDING GPP

WHAT IS GPP?

Generalized Pustular Psoriasis (GPP) is a rare, potentially life-threatening neutrophilic skin disease1 that is distinct from Plaque Psoriasis.2 Caused by a dysregulation of the IL36- pathway.3 GPP is characterised by the widespread eruption of painful, sterile pustules that appear all over the body.2 These pustules can occur with or without systemic inflammation.3

WHAT DOES GPP LOOK LIKE?4

Generalized Pustular Psoriasis (GPP) is characterised by neutrophilic pustules that coalesce to form "lakes of pus" on the skin.2

Image of GPP

 

WHAT COMORBIDITIES ARE ASSOCIATED WITH GPP? 

Comorbidities may include:

  • Psoriatic arthritis5
  • Diabetes5
  • Hyperlipidaemia6
  • Obesity5
  • Chronic obstructive pulmonary disease5
  • Chronic kidney disease6
  • Anxiety6
  • Depression6
GPP is a rare, neutrophilic skin disease characterised by episodes of
widespread eruption of sterile, macroscopically visible pustules that can
occur with or without systemic inflammation, and with or without Plaque
Psoriasis.”1

GPP IS NOT PLAQUE PSORIASIS7

Diagnosis of GPP can be difficult, and misdiagnosis is frequent. A study conducted by HealthiVibe found that 59% of patients with GPP were initially misdiagnosed. It is important to know how to differentiate GPP from other conditions such as Plaque Psoriasis.7

GPP IS PHENOTYPICALLY, HISTOPATHOLOGICALLY, AND GENETICALLY DISTINCT FROM PLAQUE PSORIASIS5

GPP impacts patients' activities of daily living. Results from a survey, that assessed the impact of GPP from the Perspective of People Living with the Condition, showed that a high percentage of patients suffered from itching, fatigue and pain.7

PATIENT-REPORTED SYMPTOM MEASURES (MEDIUM VAS SCORE, RANGE 1-100) FOR GPP AND PLAQUE PSORIASIS8

GPP Symptoms
Egypt GPP Navigation Arrow

GPP= Generalized Pustular Psoriasis; IL36-=interleukin36-; PGA=Physician Global Assessment; VAS=Visual Analogue Scale.

REFERENCES

  1. Choon SE, Lebwohl MG, Marrakchi S, et al. Study protocol of the global Effisayil 1 Phase II, multicentre, randomised, double-blind, placebo-controlled trial of spesolimab in patients with generalized pustular psoriasis presenting with an acute flare. BMJ Open. 2021;11(3):e043666

  2. Gooderham MJ, Van Voorhees AS, Lebwohl MG. An update on generalized pustular psoriasis. Expert Rev Clin Immunol. 2019;15(9):907-919.

  3. Marrakchi S, Puig L. Pathophysiology of Generalized Pustular Psoriasis. Am J Clin Dermatol. 2022;23:13-19.

  4. Marrakchi S, Guigue P, Renshaw BR, et al. Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med. 2011;365(7):620-628.

  5. Zheng M, Jullien D, Eyerich K. The Prevalence and Disease Characteristics of Generalized Pustular Psoriasis. Am J Clin Dermatol. 2022;23:5-12.

  6. Prinz JC, Choon SE, Griffiths CEM, et al. Prevalence, comorbidities and mortality of generalized pustular psoriasis: A literature review [published online ahead of print, 2022 Nov 4]. J Eur Acad Dermatol Venereol. 2022;10.1111/jdv.18720.

  7. Reisner DV, Johnsson FD, Kotowsky N, Brunette S, Valdecantos W, Eyerich K. Impact of Generalized Pustular Psoriasis from the Perspective of People Living with the Condition: Results of an Online Survey. Am J Clin Dermatol. 2022;23:65-71.

  8. Lebwohl M, Medeiros RA, Mackey RH, et al. The Disease Burden of Generalized Pustular Psoriasis: Real-World Evidence From CorEvitas’ Psoriasis Registry. Sage-Journal of Psoriasis and Psoriatic Arthritis. 2022;7(2):71-78.