«ΠΕΡΙΛΗΨΗ ΤΩΝ ΧΑΡΑΚΤΗΡΙΣΤΙΚΩΝ ΤΟΥ ΠΡΟΪΟΝΤΟΣ»

GPP is a chronic, systemic, neutrophilic inflammatory disease^2–4

Generalized pustular psoriasis (GPP), originally described by Leopold von Zumbusch in 1910, is a chronic, systemic, inflammatory skin disease with recurring flares.^2–5

A GPP flare is characterized by the sudden onset of rapidly disseminating erythema covered with sterile pustules, crusts and scales, associated with systemic symptoms.⁶

Many patients with GPP experience chronic symptoms between flares, which can include persistent scaling, crusting, and/or erythema.^6,7

GPP is distinct from plaque psoriasis^2,8,9

GPP^2,8^–¹⁰
Rare and potentially life-threatening Rapid onset Systemic symptoms are common Relapsing and persistent skin disease Innate immune inflammation Driven by the IL-36 pathway

Plaque psoriasis^2,8^–¹³
Common Gradual onset Systemic symptoms are rare Persistent skin disease Adaptive immune response Driven by the IL-23/IL-17 pathway

GPP, generalized pustular psoriasis; IL, interleukin.

GPP is unpredictable and highly heterogeneous^4,5,14

Driven by the IL-36 pathway, GPP has a heterogeneous and unpredictable clinical course, with chronic symptoms and periods of flaring.^{2–5,8,10,14}

Generalized pustular psoriasis flare and Chronic symptoms

GPP has a very large impact on patients’ QoL, even when they are not flaring^16,17

Impact of generalized pustular psoriasis on patient QoL

*
The mean DLQI was assessed in 64 patients who were no longer experiencing a GPP flare during the last follow-up in a retrospective study of 102 patients with adult-onset GPP seen in Johor, Malaysia.¹⁶

DLQI, Dermatology Quality of Life Index; GPP, generalized pustular psoriasis; QoL, quality of life.

A global Delphi consensus recommends that GPP treatment should address the chronic, inflammatory and heterogeneous nature of GPP and be well tolerated.^6†

†
A panelist of 21 dermatologists provided global opinions (Asia, Europe, USA, South America and Africa) on four key domains related to GPP; clinical course and flare definition, diagnosis, treatment goals, and holistic management.⁶

References

Reisner DV, Johnsson FD, Kotowsky N, et al. 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(Suppl 1):65–71.
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.
Lebwohl M, Medeiros RA, Mackey RH, et al. The disease burden of generalized pustular psoriasis: real-world evidence from CorEvitas’ Psoriasis Registry. J Psoriasis Psoriatic Arthritis. 2022;7(2):71–78.
Rivera-Díaz R, Daudén E, Carrascosa JM, et al. Generalized pustular psoriasis: a review on clinical characteristics, diagnosis, and treatment. Dermatol Ther (Heidelb). 2023;(3):673–688.
Choon SE, Navarini AA, Pinter A. Clinical course and characteristics of generalized pustular psoriasis. Am J Clin Dermatol. 2022;23(Suppl 1):21–29.
Puig L, Choon SE, Gottlieb AB, et al. Generalized pustular psoriasis: a global Delphi consensus on clinical course, diagnosis, treatment goals and disease management. J Eur Acad Dermatol Venereol. 2023;37(4):737–752.
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.
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.
Liang Y, Sarkar MK, Tsoi LC, Gudjonsson JE. Psoriasis: a mixed autoimmune and autoinflammatory disease. Curr Opin Immunol. 2017;49:1–8.
Gooderham MJ, Van Voorhees AS, Lebwohl MG. An update on generalized pustular psoriasis. Expert Rev Clin Immunol. 2019;15(9):907–919.
Langley RGB, Krueger GG, Griffiths CEM. Psoriasis: epidemiology, clinical features, and quality of life. Ann Rheum Dis. 2005;64(suppl 2):ii18–ii23.
Burden AD, Kirby B. Psoriasis and related disorders. Griffiths C, et al, eds. In: Rook’s Textbook of Dermatology. 9th ed. Blackwell, UK: Wiley; 2016.
WHO Report 2016. Available at: https://apps.who.int/iris/handle/10665/204417. Accessed September 2024.
Ly K, Beck KM, Smith MP, et al. Diagnosis and screening of patients with generalized pustular psoriasis. Psoriasis (Auckl). 2019;9:37–42.
Burden AD, Bachelez H, Choon SE, al. The Generalized Pustular Psoriasis Physician Global Assessment (GPPGA) score: online assessment and validation study of a specific measure of GPP disease activity. Br J Dermatol. 2023;189(1):138–140.
Choon SE, Lai N M, Mohammad NA, et al. 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.
Dermatology Quality of Life Index (DLQI). Available at: https://www.cardiff.ac.uk/medicine/resources/quality-of-life-questionnaires/dermatology-life-quality-index. Accessed September 2024.

GPP (07/2025) PC-GR-102302

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GPP can impose a substantial burden on patients