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UNDERSTANDING GPP

WHAT IS GPP?

Generalized Pustular Psoriasis (GPP) is a rare, potentially life-threatening neutrophilic skin disease that is distinct from Plaque Psoriasis. Caused by a dysregulation of the IL-36 pathway, GPP is characterised by the widespread eruption of painful, sterile pustules that appear all over the body. These pustules can occur with or without systemic inflammation.1-5

WHAT DOES GPP LOOK LIKE?

Generalized Pustular Psoriasis (GPP) is characterised by neutrophilic pustules that coalesce to form "lakes of pus" on the skin.1,6

Image of GPP

 

WHAT COMORBIDITIES ARE ASSOCIATED WITH GPP? 

Comorbidities may include7:

  • Psoriatic arthritis
  • Diabetes
  • Hyperlipidaemia
  • Obesity
  • Chronic obstructive pulmonary disease
  • Chronic kidney disease
  • Anxiety
  • Depression

DR STROBER PROVIDES MORE INSIGHT ON GPP

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.”
Dr Bruce Strober

GPP IS NOT PLAQUE PSORIASIS

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.2,8,9

GPP IS PHENOTYPICALLY, HISTOPATHOLOGICALLY, AND GENETICALLY DISTINCT FROM PLAQUE PSORIASIS2,3

GPP has a greater effect on patients’ quality of life than Plaque Psoriasis. Results from a survey using the CorEvitas Psoriasis Registry showed that median patient-reported pain, itching, and fatigue were higher in GPP than in Plaque Psoriasis.10

Additionally, while Plaque Psoriasis is associated with an increased risk of cardiovascular disease, it is not associated with life-threatening complications and does not commonly necessitate hospitalisation.11,12

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

GPP Symptoms

DR GOODERHAM AND DR LANGLEY PROVIDE MORE INSIGHT ON THE DIFFERENCES BETWEEN GPP AND PLAQUE PSORIASIS

Even during the quiescent phase, the impact of GPP on the quality of life is greater than with Plaque Psoriasis.”
Dr Melinda Gooderham

IDENTIFY THE DIFFERENCES BETWEEN GPP AND PLAQUE PSORIASIS13-16

Select the skin disorder that matches the disease characteristic.

Innate immune inflammation

Neutrophilic

Adaptive immune response

Autoimmune disease

Driven by IL-36 pathway

Driven by IL-23 pathway

Associated with genetic mutations affecting IL-36

IL-23=interleukin-23; IL-36=interleukin-36; 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. doi:10.1136/bmjopen-2020-043666

  2. Navarini AA, Burden AD, Capon F, et al; for the ERASPEN Network. European consensus statement on phenotypes of pustular psoriasis. J Eur Acad Dermatol Venereol. 2017;31(11):1792-1799. doi:10.1111/jdv.14386 

  3. Benjegerdes KE, Hyde K, Kivelevitch D, Mansouri B. Pustular psoriasis: pathophysiology and current treatment perspectives. Psoriasis (Auckl). 2016;6:131-144. doi:10.2147/PTT.S98954 

  4. Gabay C, Towne JE. Regulation and function of interleukin-36 cytokines in homeostasis and pathological conditions. J Leukoc Biol. 2015;97(4):645-652. doi:10.1189/jlb.3RI1014-495R

  5. Marrakchi S, Guigue P, Renshaw BR, et al. Interleukin-36–receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med. 2011;365(77):620-628. doi:10.1056/NEJMoa1013068 

  6. Ly K, Beck KM, Smith MP, Thibodeaux Q, Bhutani T. Diagnosis and screening of patients with generalized pustular psoriasis. Psoriasis (Auckl). 2019;9:37-42. doi:10.2147/PTT.S181808 

  7. Kotowsky N, Feldman S, Garry EM, Valdecantos WC, Gao R, Golembesky AK. Characteristics of patients with generalized pustular psoriasis compared with those with psoriasis vulgaris: a claims database study. Poster presented at: 16th Annual Maui Derm for Dermatologists; January 25-29, 2020; Maui, Hawaii. 

  8. Strober B, Leman J, Mockenhaupt M, et al. Unmet educational needs and clinical practice gaps in the management of generalized pustular psoriasis: global perspectives from the front line. Poster presented at: 6th World Psoriasis & Psoriatic Arthritis Conference; June 30-July 3, 2021; Stockholm, Sweden.

  9. Kotowsky N, Brunette SR, Valdecantos WC. Insights from patients with generalized pustular psoriasis: results of an online survey in the USA. Poster presented at: 30th European Academy of Dermatology and Venereology Congress; September 29-October 2, 2021; virtual. 

  10. Strober B, Kotowsky N, Medeiros RA, et al. Patient-reported outcomes from a large, North American-based cohort highlight a greater disease burden for generalized pustular psoriasis versus plaque psoriasis: real-world evidence from the Corrona Psoriasis Registry. Poster presented at: American Academy of Dermatology Virtual Meeting Experience; April 23-25, 2021. 

  11. Prey S, Paul C, Bronsard V, et al. Cardiovascular risk factors in patients with plaque psoriasis: a systematic review of epidemiological studies. J Eur Acad Dermatol Venereol. 2010;24(suppl 2):23-30. doi:10.1111/j.1468-3083.2009.03564.x

  12. Pardasani AG, Feldman SR, Clark AR. Treatment of psoriasis: an algorithm-based approach for primary care physicians. Am Fam Physician. 2000;61(3):725-733.  

  13. Johnston A, Xing X, Wolterink L, et al. IL-1 and IL-36 are dominant cytokines in generalized pustular psoriasis. J Allergy Clin Immunol. 2017;140(1):109-120. doi:10.1016/j.jaci.2016.08.056 

  14. Liang Y, Sarkar MK, Tsoi LC, Gudjonsson JE. Psoriasis: a mixed autoimmune and autoinflammatory disease. Curr Opin Immunol. 2017;49:1-8. doi:10.1016/j.coi.2017.07.007 

  15. Gooderham MJ, Van Voorhees AS, Lebwohl MG. An update on generalized pustular psoriasis. Expert Rev Clin Immunol. 2019;15(9):907-919. doi:10.1080/1744666X.2019.1648209 

  16. Furue K, Yamamura K, Tsuji G, et al. Highlighting interleukin-36 signalling in plaque psoriasis and pustular psoriasis. Acta Derm Venereol. 2018;98(1):5-13. doi:10.2340/00015555-2808