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Understanding GPP

UNDERSTANDING GPP

WHAT IS GPP?

GPP IS A RARE, CHRONIC DISEASE DISTINCT FROM PLAQUE PSORIASIS1-4

Generalized pustular psoriasis (GPP) is heterogeneous and can be a relapsing disease with recurrent flares or a persistent disease with intermittent flares.1,5 Characterized by innate immune inflammation, GPP is phenotypically, histopathologically, and genetically distinct from plaque psoriasis.1,2,6 GPP is considered a neutrophilic disease, whereas plaque psoriasis, characterized by adaptive responses, is considered an autoimmune disease.1,6

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.7 GPP can be associated with mutations in
the IL-36RN gene, but plaque psoriasis is not.6

GPP vs plaque psoriasis graphic

WHAT DOES GPP LOOK LIKE?

GPP FLARES CAN PRESENT WITH SYSTEMIC SIGNS AND SYMPTOMS, INCLUDING:

Sterile pustules, pain, scaling, erythema, dryness, itching, burning, swelling, malaise, joint pain, leukocytosis, headache, fever, and fatigue3,5,9,10

gpp flare on chest and abdomen

MICROSCOPIC AND MACROSCOPIC FEATURES OF GPP. HISTOPATHOLOGY OF PSORIASIS.11

microscopic features of gpp

(a) Psoriasis vulgaris characteristically shows acanthosis, parakeratosis, and dermal inflammatory infiltrates.

macroscopic features of gpp

(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(6):1475. doi:10.3390/ijms20061475] licensed under CC-BY 4.0).

WHAT TRIGGERS GPP?

Recognizing what triggers flares early may help you manage your patients' disease. GPP can be triggered by3,12:

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SUNLIGHT
upper respiratory tract infections icon

UPPER RESPIRATORY TRACT INFECTIONS
medications icon

CERTAIN MEDICATIONS
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SUDDEN WITHDRAWAL OF STEROIDS
stress icon

STRESS
infection icon

INFECTION
pregnancy icon

PREGNANCY

LEARN THE SIGNS, SYMPTOMS, AND COMORBIDITIES OF GPP

READY TO LEARN ABOUT THE PATHOGENESIS OF GPP?

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The pathology of GPP

IL-17=interleukin-17; IL-22=interleukin-22; IL-23=interleukin-23; IL-36=interleukin-36; IL-RAcP=interleukin-1 receptor accessory protein; IL-36RN=interleukin-36 receptor antagonist; TIR=toll/interleukin-1 receptor; TNF-α=tumor necrosis factor alpha.

REFERENCES

  1. 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

  2. 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

  3. 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

  4. Akiyama M, Takeichi T, McGrath JA, Sugiura K. Autoinflammatory keratinization diseases: an emerging concept encompassing various inflammatory keratinization disorders of the skin. J Dermatol Sci. 2018;90(2):105-111. doi:10.1016/j.jdermsci.2018.01.012

  5. Bachelez H. Pustular psoriasis and related pustular skin diseases. Br J Dermatol. 2018;178(3):614-618. doi:10.1111/bjd.16232

  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. 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

  8. 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

  9. 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

  10. Shah M, Al Aboud DM, Crane JS, Kumar S. Pustular psoriasis. StatPearls. Updated August 8, 2022. Accessed March 30, 2023. https://www.ncbi.nlm.nih.gov/books/NBK537002/

  11. Rendon A, Schäkel K. Psoriasis pathogenesis and treatment. Int J Mol Sci. 2019;20(6)1475. doi:10.3390/ijms20061475

  12. 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