WHAT IS GPP?

Generalised Pustular Psoriasis (GPP) is a severe and potentially life-threatening neutrophilic skin disease characterised by episodes of widespread eruption of sterile, macroscopically visible pustules that occurs frequently with systemic inflammation.¹ GPP can occur with or without plaque psoriasis, and the concurrence rates of up to 54.4% have been reported in GPP patients in the UK/Ireland.²

LEARN ABOUT GPP: WHAT'S IN A NAME

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GPP: What's in a name

Speaker: Prof. Alexander Navarini

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WHAT DOES GPP LOOK LIKE?

The clinical course of GPP is heterogeneous and can be characterised as a relapsing disease with recurrent flares with inter-flare periods of little to no activity, or a persistent disease with intermittent flares.^1-3

GPP FLARES

GPP flares, also referred to as acute GPP, is characterised by neutrophilic pustules that coalesce to form lakes of pus on the skin. Flares can present with systemic symptoms (fever, increased C-reactive protein (CRP) and neutrophilia).^3-6

(A)

(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 what triggers flares early may help you manage your patients' disease. GPP can be triggered by^4-7:

SUNLIGHT

UPPER RESPIRATORY TRACT INFECTIONS

CERTAIN MEDICATIONS

SUDDEN WITHDRAWAL OF STEROIDS

STRESS

INFECTION

PREGNANCY

PAIN

WHAT COMORBIDITIES ARE ASSOCIATED WITH GPP?

Comorbidities may include^6-8:

Psoriatic arthritis
Diabetes
Hyperlipidemia
Obesity
Chronic obstructive pulmonary disease
Chronic kidney disease
Anxiety
Depression

REFERENCES

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
Twelves S, Mostafa A, Dand N et al. Clinical and genetic differences between pustular psoriasis subtypes. J Allergy Clin Immunol. 2019 Mar;143(3):1021-1026. doi: 10.1016/j.jaci.2018.06.038
Bachelez H. Pustular psoriasis: the dawn of a new era. Acta Derm Venereol. 2020;100(3):adv00034. doi:10.2340/00015555-3388
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
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
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
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
Prinz JC, Choon SE, Griffiths CEM et al. Prevalence, comorbidities and mortality of generalized pustular psoriasis: A literature review. J Eur Acad Dermatol Venereol. 2023 Feb;37(2):256-273. doi: 10.1111/jdv.18720

SC-GB-01617, May 2023

Understanding GPP