Generalized pustular psoriasis

Authors

  • Lara Constanza Marotte Francisco Javier Muñiz Infectious Disease Hospital, City of Buenos Aires, Argentina
  • María Emilia Candiz Francisco Javier Muñiz Infectious Disease Hospital, City of Buenos Aires, Argentina
  • Olga Forero Francisco Javier Muñiz Infectious Disease Hospital, City of Buenos Aires, Argentina
  • Viviana Leiro Francisco Javier Muñiz Infectious Disease Hospital, City of Buenos Aires, Argentina

DOI:

https://doi.org/10.47196/yrgaz382

Keywords:

generalized pustular psoriasis, pathophysiology, interleukin-36, treatment

Abstract

Generalized pustular psoriasis is a rare autoinflammatory disease but is considered a dermatological emergency. It presents as an acute and severe systemic condition, with flares characterized by sterile pustules and significant systemic involvement. It is currently regarded as a distinct entity with a genetic and pathogenic profile different from plaque psoriasis. Early diagnosis and therapeutic management are essential. The aim of this publication was to provide an updated review of the condition, with a focus on therapeutic options.

Author Biographies

  • Lara Constanza Marotte, Francisco Javier Muñiz Infectious Disease Hospital, City of Buenos Aires, Argentina

    Dermatologist, Chief Resident, Dermatology Department

  • María Emilia Candiz, Francisco Javier Muñiz Infectious Disease Hospital, City of Buenos Aires, Argentina

    Dermatologist, Staff Physician in charge of the Psoriasis Clinic

  • Olga Forero, Francisco Javier Muñiz Infectious Disease Hospital, City of Buenos Aires, Argentina

    Dermatologist, Staff Physician in charge of the Psoriasis Clinic

  • Viviana Leiro, Francisco Javier Muñiz Infectious Disease Hospital, City of Buenos Aires, Argentina

    Dermatologist, Head of the Dermatology Unit

References

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

II. Choon SE, Navarini AA, Pinter A. Clinical course and characteristics of generalized pustular psoriasis. Am J Clin Dermatol. 2022;23:21-29.

III. Bachelez H, Barker J, Burden AD, Navarini AA, et al. Generalized pustular psoriasis is a disease distinct from psoriasis vulgaris: evidence and expert opinion. Expert Rev Clin Immunol. 2022;18:1033-1047.

IV. Romiti R, Hirayama AL, Arnone M, Magalhães RF. Generalized pustular psoriasis (von Zumbusch). An Bras Dermatol. 2022;97:63-74.

V. Navarini AA, Burden AD, Capon F, Mrowietz U, et al. European consensus statement on phenotypes of pustular psoriasis. J Eur Acad Dermatol Venereol. 2017;31:1792-1799.

VI. Rivera-Díaz R, Daudén E, Carrascosa JM, Cueva P, et al. A review on clinical characteristics, diagnosis, and treatment. Dermatol Ther (Heidelb). 2023;13:673-688.

VII. Zheng M, Jullien D, Eyerich K. The prevalence and disease characteristics of generalized pustular psoriasis. Am J Clin Dermatol. 2022;23:5-12.

VIII. Markeson CD, Huang S, Hsu S. Eruption of generalized pustular psoriasis following initiation of adalimumab for hidradenitis suppurativa: a case report. Cureus. 2024;16:e75402.

IX. Wang Y, Yang F, Wang R, Luo S. Paradoxical psoriasis induced by IL-17 antagonists. Indian J Dermatol Venereol Leprol. 2024;90:623-631.

X. Ly K, Beck KM, Smith MP, Thibodeaux Q, et al. Diagnosis and screening of patients with generalized pustular psoriasis. Psoriasis (Auckl). 2019;9:37-42.

XI. Fujita H, Terui T, Hayama K, Akiyama M, et al. Japanese guidelines for the management and treatment of generalized pustular psoriasis: the new pathogenesis and treatment of GPP. J Dermatol. 2018;45:1235-1270.

XII. Burden AD, Bissonnette R, Lebwohl MG, Gloede T, et al. Psychometric validation of the generalized pustular psoriasis physician global assessment (GPPGA) and generalized pustular psoriasis area and severity index (GPPASI). J Eur Acad Dermatol Venereol. 2023;37:1327-1335.

XIII. Burden AD, Choon SE, Gottlieb AB, Navarini AA, et al. Clinical disease measures in generalized pustular psoriasis. Am J Clin Dermatol. 2022;23:39-50.

XIV. Kearns DG, Chat VS, Zang PD, Han G, et al. Review of treatments for generalized pustular psoriasis. J Dermatolog Treat. 2021;32:492-494.

XV. Bachelez H, Choon SE, Marrakchi S, Burden AD, et al. Trial of spesolimab for generalized pustular psoriasis. N Engl J Med. 2021;385:2431-2440.

XVI. Boehner A, Navarini AA, Eyerich K. Generalized pustular psoriasis. A model disease for specific targeted immunotherapy: systematic review. Exp Dermatol. 2018;27:1067-1077.

XVII. Okubo Y, Umezawa Y, Sakurai S, Hoshii N, et al. Efficacy and safety of certolizumab pegol in Japanese patients with generalized pustular psoriasis and erythrodermic psoriasis: 52-week results. Dermatol Ther (Heidelb). 2022;12:1397-1415.

XVIII. Yamanaka K, Okubo Y, Yasuda I, Saito N, et al. Efficacy and safety of risankizumab in Japanese patients with generalized pustular psoriasis or erythrodermic psoriasis: primary analysis and 180-week follow-up results from the phase 3, multicenter IMMspire study. J Dermatol. 2023;50:195-202.

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Published

2026-07-16

Issue

Section

Continuing Medical Education