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UNDERSTANDING GPP

What is GPP?

GPP is a rare disease distinct from plaque psoriasis1-3

Generalized Pustular Psoriasis (GPP) is heterogenous and can be a relapsing disease with recurrent flares or a persistent disease with intermittent flares.2,4 Characterised by innate immune inflammation, GPP is phenotypically, histopathologically, and genetically distinct from Plaque Psoriasis.2,3,5,6 GPP is considered a neutrophilic disease, whereas Plaque Psoriasis, characterised by adaptive responses, is considered an autoimmune disease.5-7

Plaque Psoriasis biomarkers tend to overlap with those of GPP, including the TNF-α/IL-23/IL-17/IL-22/IL-36 axis; however, the IL-36 pathway drives the pathogenesis of GPP, while the IL-23 axis drives Plaque Psoriasis.8 GPP can be associated with mutations in the IL36RN gene, but Plaque Psoriasis is not.6

GPP_PSO_characteristics

WHAT DOES GPP LOOK LIKE?

GPP FLARES CAN PRESENT WITH SYSTEMIC SIGNS AND SYMPTOMS: Sterile pustules, pain, scaling, erythema, dryness, itching, burning, swelling, malaise, joint pain, leukocytosis, headache, fever, and fatigue4,9-12

Image of a generalized pustular psoriasis flare across a patient’s back
image of psoriasis vulgaris under a microscope

(A) 

image of generalized pustular psoriasis (GPP) under a microscope

(B) 

Microscopic and macroscopic features of GPP. Histopathology of psoriasis. (A) Psoriasis vulgaris characteristically shows acanthosis, parakeratosis, and dermal inflammatory infiltrates. (B) In pustular psoriasis, acanthotic changes are accompanied by epidermal predominantly neutrophilic infiltrates, which cause pustule formation. (Reproduced from “Psoriasis Pathogenesis and Treatment” by Adriana Rendon and Knut Schäkel [Int. J. Mol. Sci. 2019; 20:1475; http://dx.doi.org/10.3390/ijms20061475 ] licensed under CC-BY 4.0). 

WHAT TRIGGERS GPP?

Recognising the triggers of flares early may help you manage your patients' disease. GPP can be triggered by9,13:

Sunlight

SUNLIGHT

Upper respiratory infections

UPPER RESPIRATORY TRACT INFECTIONS

Certain medication

CERTAIN MEDICATIONS

Sudden withdrawal of steroids

SUDDEN WITHDRAWAL OF STEROIDS

Stress

STRESS

Infection

INFECTION

Pregnancy

PREGNANCY

LEARN ABOUT THE SIGNS, SYMPTOMS, AND COMORBIDITIES OF GPP

Ready to learn about the pathogenesis of GPP?

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Pathology of GPP Video
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.
    Bachelez H. Pustular psoriasis: the dawn of a new era. Acta Derm Venereol. 2020;100(3):adv00034. doi:10.2340/00015555-3388
  • 5.
    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
  • 6.
    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
  • 7.
    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
  • 8.
    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
  • 9.
    Strober B, Kotowsky N, Medeiros R, et al. 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;11(2):529-541. doi:10.1007/s13555-021-00493-0
  • 10.
    Skalicky A, Rentz A, Esser D, Thoma C, Gloede T. Symptom experience of patients with generalized pustular psoriasis (GPP). Value in Health. 2020;23(suppl 1):S345. Abstract: PRO89. doi:10.1016/j.jval.2020.04.1310
  • 11.
    Choon SE, Lai NM, Mohammad NA, Nanu NM, Tey KE, Chew SF. Clinical profile, morbidity, and outcome of adult-onset generalized pustular psoriasis: analysis of 102 cases seen in a tertiary hospital in Johor, Malaysia. Int J Dermatol. 2014;53(6):676-684. doi:10.1111/ijd.12070
  • 12.
    Shah M, Al Aboud DM, Crane JS, Kumar S. Pustular psoriasis. StatPearls. Updated August 10, 2020. Accessed June 2, 2021. https://www.ncbi.nlm.nih.gov/books/NBK537002/
  • 13.
    Kharawala S, Golembesky AK, Bohn RL, Esser D. The clinical, humanistic, and economic burden of generalized pustular psoriasis: a structured review. Expert Rev Clin Immunol. 2020;16(3):239-252. doi:10.1080/1744666X.2019.1708193