Spesolimab for treating acute generalised pustular psoriasis [ID3963]

Psoriasis is an inflammatory skin disease that typically follows a relapsing and remitting course. The most common form of psoriasis is plaque psoriasis which is characterised by raised plaques on the skin.

Generalised pustular psoriasis (GPP) also known as von Zumbusch psoriasis is a rare form of psoriasis characterised by recurrent episodes of widespread eruption of pustules.

This leads to large surface areas of the skin becoming inflamed red and developing pustules over the body specifically in the bends or folds of the skin genital regions and fingertips. GPP can be life threatening if left untreated as it can lead to organ failure.

Although it is a chronic and persistent condition its course may be unpredictable with flare-ups and remissions. (123)Historically GPP has been classified as a variant of psoriasis consequently assessment and treatment have commonly involved elements from guidance developed for plaque psoriasis.

Evidence from genetic histological immunological and clinical studies suggest that GPP is a distinct disease as it has unique skin and systemic symptoms.(3) The genetic mutations commonly associated with the development of this condition are IL36RN and CARD14.

Alteration in these genes can trigger overly active signalling pathways that promote skin inflammation.(4)The prevalence of GPP in England is unclear.

A French study estimated a prevalence of 1.76 patients per million while a study from Japan estimated a prevalence of 7.46 patients per million.

Estimating the number of people affected by GPP is difficult because rare diseases are often misdiagnosed and patient numbers are small. (56)There are no specific NICE guidelines for generalised pustular psoriasis.

Suspected generalised pustular psoriasis flares should be managed as a medical emergency and immediate same-day specialist dermatology assessment must be arranged.

NICE clinical guideline 153 on psoriasis in general recommends that people withpsoriasis should be offered topical therapies such as corticosteroids systemic nonbiological therapies and systemic biological therapies.

Some of the treatments recommended for other types of psoriasis might be used for generalised pustular psoriasis flares. However there are currently no licensed treatments specifically for this condition.

In clinical practice this condition is normally managed in hospital care where topical therapy is provided as well as systemic non biological therapy such as ciclosporin and biological therapies from the IL-17 and 23 family inhibitors.References:1. Psoriasis- symptoms NHS (2022) available from: https://www.nhs.uk [Accessed June 2024]2.

Generalised Pustular Psoriasis.

Psoriasis treatment (2015) available from: https://patient.info/doctor/generalised-pustular-psoriasis [Accessed June 2024]3.

Burden D Eng Choon S Gottlieb A Navarini A and Warren R (2022) Clinical Disease Measures in Generalized Pustular Psoriasis. American Journal ofDermatology pp.39-50.4.

Generalized pustular psoriasis NIH Medline plus (2017) available from: https://medlineplus.gov/genetics/condition/generalized-pustularpsoriasis/#causes [Accessed June 2024]5. Augey F Renaudier P Nicolas J (2006) Generalized pustular psoriasis (Zumbusch): a French epidemiological survey. European Journal ofDermatology 16(6):669-73.6.

Zheng M Jullien D and Eyerich K (2022) The prevalence and disease characteristics of generalized pustular psoriasis. American Journal ofDermatology pp.5-12.