What is GPP?

GPP is a serious, chronic, and heterogeneous autoinflammatory condition^1,2

GPP is characterized by sudden, painful flares that can impose a substantial burden on patients, even between flares.^1,3

Patients with GPP may continue to experience skin and systemic symptoms.^1,4

GPP flare frequency, severity, and duration vary between patients and between flares.²

Hear from a GPP expert

GPP during a flare⁵

Skin with an ongoing GPP flare up

During a flare, GPP skin symptoms may cover as little as
5% body surface area (BSA).⁶

GPP between flares⁵

GPP between flare ups

Between flares, GPP skin symptoms may include scaling,
erythema, and pustules.⁷

GPP may have a serious impact on the patient even when they are not flaring³

In a post hoc analysis, evaluating disease burden in untreated GPP patients, GPP negatively impacted patients, even in the absence of a flare.⁸

47^%

of non-flaring patients experienced

moderate pain and symptoms^a,b

20^%

of non-flaring patients experienced

severe pain^a

13^%

of non-flaring patients experienced

severe symptoms^b

Post hoc analyses, even those based on observations from clinical trials, are not intended to be compared with results from prespecified analyses of controlled clinical trials. Differences in patient populations, outcome definitions, and methods of collecting data make it inappropriate to compare this analysis to controlled clinical trial outcome data. Data from a post hoc analysis should be viewed as relevant supplementary information. No information from this post hoc analysis should be in any way construed as making any claim about the efficacy, safety, or appropriateness of any specific therapy.

GPP, generalized pustular psoriasis; GPPPGA, Generalized Pustular Psoriasis Physician Global Assessment; PSS, Psoriasis Symptoms Scale; VAS, Visual Analogue Scale.

^aPatient-reported severity of pain was evaluated using the Pain VAS score (continuous scale 0-100; 0-4=no pain, 5-44=mild pain, 45-74=moderate pain, 75-100=severe pain).⁸

^bPatient-reported severity of GPP symptoms was evaluated using the PSS score (0=no symptoms, 4=mild symptoms, 8=moderate symptoms, 12=severe symptoms, 16=very severe symptoms).⁸

GPP burden may continue beyond the flare¹

GPP symptoms may include^2,4,7:

Itching, burning, and pain
Erythema
Pustular lesions
Skin scaling and crusting
Joint pain

Disabling edema
Malaise
Fatigue
Fever
Chills

Treating and managing GPP starts with timely assessment⁹

The Generalized Pustular Psoriasis Physician Global Assessment (GPPPGA) is used to assess GPP symptoms and evaluate treatment efficacy.⁹

GPP assessment visual tool

Download the GPPPGA tool

The GPPPGA total score ranges from 0 (clear) to 4 (severe) and is determined by averaging the subscores of pustules, erythema, and scaling. These components are graded separately. The average is calculated: 0^a (0 for all 3 components), 1^a (average is >0 to <1.5), 2 (average is 1.5 to <2.5), 3 (average is 2.5 to <3.5), or 4 (average is ≥3.5).^9,10

GPP, generalized pustular psoriasis; GPPPGA, Generalized Pustular Psoriasis Physician Global Assessment.

^aTo receive a score of 0 or 1, the patient should be afebrile, in addition to skin presentation requirements.¹⁰

FAQ

Why is it important to treat GPP continuously?

Learn more by watching this video

Watch expert Dr. Adam Friedman, Professor and Chair of Dermatology at George Washington University School of Medicine and Health Sciences, discuss treatment goals and the importance of continuous GPP treatment.

Please see Important Safety Information below.

Dr. Adam Friedman has been compensated for his time.

Transcript

Generalized pustular psoriasis, or GPP, is a chronic disease with a high patient burden. GPP is also unpredictable, which means there's always a possibility of GPP flares because flares can happen at any time. GPP flares usually involve skin symptoms such as pustules, scaling, erythema. But systemic symptoms are common too. These can include fever, malaise, fatigue, joint pain, and even edema.

Between the flares, patients with GPP may also experience ongoing chronic skin symptoms. These can include scaling, crusting, and even erythema. In addition, patients may experience ongoing chronic symptoms such as fever, malaise, fatigue, joint pain, and edema.

For my GPP patients my goals are first, treat any acute flare, second, reduce the risk of a future flare and of course, manage the chronic symptoms of the disease.

SPEVIGO is the only FDA-approved treatment that addresses the acute and chronic symptoms of GPP. Evidence from the Effisayil® 2 clinical study demonstrates that continuous subcutaneous treatment with SPEVIGO may help reduce the risk of a future GPP flare. In this clinical trial, SPEVIGO SC significantly reduced the risk of GPP flares by 84% for at least 48 weeks versus placebo.

Of note, three patients experienced a GPP flare in the first 4 weeks after 1 dose of SPEVIGO SC treatment. No patients experienced a flare in the SPEVIGO arm of the trial after week 4 through the end of the trial at week 48.

The National Psoriasis Foundation advocates that GPP
patients need timely access to FDA-approved therapies⁹

FDA, US Food and Drug Administration; GPP, generalized pustular psoriasis.

Learn how SPEVIGO works to treat all phases of GPP

Explore MOA

Indication

SPEVIGO is indicated for the treatment of generalized pustular psoriasis (GPP) in adults and pediatric patients 12 years of age and older and weighing at least 40 kg (88 lb).

Please see SPEVIGO Prescribing Information, including Medication Guide.

Important Safety Information

Contraindications

SPEVIGO is contraindicated in patients with severe or life-threatening hypersensitivity to spesolimab-sbzo or to any of the excipients in SPEVIGO. Reported hypersensitivity reactions have included drug reaction with eosinophilia and systemic symptoms (DRESS) and anaphylaxis.

WARNINGS AND PRECAUTIONS

Infections: SPEVIGO may increase the risk of infections. In patients with a chronic infection or a history of recurrent infection, consider the potential risks and expected clinical benefits of treatment prior to prescribing SPEVIGO. Treatment with SPEVIGO is not recommended in patients with any clinically important active infection until the infection resolves or is adequately treated. Instruct patients to seek medical advice if signs or symptoms of clinically important infection occur during or after treatment with SPEVIGO. If a patient develops a clinically important active infection, discontinue SPEVIGO therapy until the infection resolves or is adequately treated.

Risk of Tuberculosis: Evaluate patients for tuberculosis (TB) infection prior to initiating treatment with SPEVIGO. Avoid use of SPEVIGO in patients with active TB infection. Consider initiating anti-TB therapy prior to initiating SPEVIGO in patients with latent TB or a history of TB in whom an adequate course of treatment cannot be confirmed. Monitor patients for signs and symptoms of active TB during and after SPEVIGO treatment.

Hypersensitivity and Infusion-Related Reactions:

Serious hypersensitivity reactions, including anaphylaxis and delayed reactions such as drug reaction with eosinophilia and systemic symptoms (DRESS), have been reported during and following administration of SPEVIGO. These reactions can occur with the first dose or subsequent doses.
SPEVIGO is contraindicated in patients with severe or life-threatening hypersensitivity to spesolimab-sbzo or to any of the excipients in SPEVIGO. If a patient develops signs of anaphylaxis or other serious hypersensitivity, discontinue SPEVIGO immediately and initiate appropriate treatment.
If a patient develops mild or moderate hypersensitivity during an intravenous infusion or other infusion-related reactions, stop SPEVIGO infusion and consider appropriate medical therapy (eg, systemic antihistamines and/or corticosteroids). Upon resolution of the reaction, the infusion may be restarted at a slower infusion rate with gradual increase to complete the infusion.

Vaccinations: Prior to initiating SPEVIGO for treatment of GPP, complete all age-appropriate vaccinations according to current immunization guidelines. Avoid use of live vaccines in patients during and for at least 16 weeks after treatment with SPEVIGO. No specific studies have been conducted in SPEVIGO-treated patients who have recently received live viral or live bacterial vaccines.

ADVERSE REACTIONS

Intravenous SPEVIGO for Treatment of GPP Flare (Study Effisayil-1): Most common adverse reactions reported in ≥5% of patients treated with SPEVIGO in the clinical trial were asthenia and fatigue, headache, nausea, pruritus and prurigo, infusion site hematoma and bruising, and urinary tract infection (UTI).

Specific Adverse Reactions

Infections: The most frequent adverse reactions that occurred in subjects treated with intravenous SPEVIGO were infections. During the 1-week placebo-controlled period in Study Effisayil-1, infections were reported in 14% of subjects treated with SPEVIGO compared with 6% of subjects treated with placebo. Serious infection (UTI) was reported in 1 subject (3%) in the SPEVIGO group and no subjects in the placebo group. Infections observed through Week 1 in Study Effisayil-1 in subjects treated with SPEVIGO were mild (29%) to moderate (71%).
Drug Reaction With Eosinophilia and Systemic Symptoms (DRESS): Two cases of DRESS were reported in Study Effisayil-1 in subjects with GPP who were treated with intravenous SPEVIGO. RegiSCAR DRESS validation scoring (with the following categories: “no,” “possible,” “probable,” or “definite” DRESS) was applied to the reported cases. Reported cases were assessed as “no DRESS” and “possible DRESS.”

Subcutaneous SPEVIGO for Treatment of GPP When Not Experiencing a Flare (Study Effisayil-2): Regarding the exposure-adjusted incidence rates for subjects on randomized treatment prior to receiving rescue treatment for flare or completing trial without a flare, the rate per 100-patient years for injection site reaction (including erythema, pain, swelling, induration, urticaria, and warmth at the injection site) was 31.6 for the subcutaneous SPEVIGO cohort (600 mg loading dose followed by 300 mg every 4 weeks) vs 12.7 for the placebo cohort. The rate per 100-patient years for UTI was 18 for SPEVIGO vs 0 for placebo. The rate per 100-patient years for pruritus was 8.8 for SPEVIGO vs 0 for placebo. The rate per 100-patient years for arthralgia was 13.3 for SPEVIGO vs 6 for the placebo cohort. There were 3 subjects who discontinued subcutaneous SPEVIGO due to treatment-emergent adverse events of psoriasis compared to no subjects in the placebo cohort who discontinued placebo for any treatment-emergent adverse event.

Safety in Study Effisayil-2 After Flare: In Effisayil-2, subjects who experienced a GPP flare and received at least one dose of an open-label single intravenous 900 mg dose of SPEVIGO were treated with open-label subcutaneous SPEVIGO 300 mg. These subjects (n=19) received subcutaneous dosing at every 12 weeks, which could have been increased to every 4 weeks based on GPPPGA total score or pustulation subscore increased by ≥1 from any previous open-label maintenance visit. The reported safety profile of open-label subcutaneous SPEVIGO use after treatment of GPP flare with open-label intravenous SPEVIGO use was consistent with the safety profiles of use of SPEVIGO from Trial Effisayil-1 and randomized controlled data from Trial Effisayil-2.

Clinical Development of Spesolimab-sbzo

Guillain-Barre Syndrome (GBS): Among approximately 835 subjects exposed to spesolimab-sbzo during clinical development, GBS was reported in 3 subjects who received various doses of spesolimab-sbzo via various methods of administration in clinical trials for unapproved indications.

SPECIFIC POPULATIONS

Pediatric Use: The safety and effectiveness of SPEVIGO for the treatment of GPP have been established in pediatric patients 12 years of age and older and weighing at least 40 kg. Use of SPEVIGO for this indication is supported by data from a randomized, placebo-controlled study, which included 6 pediatric subjects 14 to 17 years of age with a history of GPP treated with subcutaneous SPEVIGO (Study Effisayil-2), and evidence from an adequate and well-controlled study of intravenous SPEVIGO in adults with GPP (Study Effisayil-1), with additional pharmacokinetic analyses showing similar drug exposure levels in adults and pediatric subjects 12 years of age and older and weighing 40 kg or more. The safety and effectiveness of SPEVIGO in pediatric patients younger than 12 years of age or in pediatric patients weighing less than 40 kg have not been established.

Please see SPEVIGO Prescribing Information, including Medication Guide.

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References

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
Choon SE, Navarini AA, Pinter A. Clinical course and characteristics of generalized pustular psoriasis. Am J Clin Dermatol. 2022;23(Suppl 1):21–29. doi:10.1007/s40257-021-00654-z
Reisner DV, Johnsson FD, Kotowsky N, Brunette S, Valdecantos W, Eyerich K. 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. doi:10.1007/s40257-021-00663-y
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
Burden AD, Mrowietz U, Skalicky AM, et al. Symptom experience and content validity of the Psoriasis Symptom Scale (PSS) in patients with generalized pustular psoriasis (GPP). Dermatol Ther (Heidelb). 2022;12(6):1367–1381. doi:10.1007/s13555-022-00736-8
Puig L, Carrascosa JM, Rivera R, et al. Generalized pustular psoriasis: review and consensus of the Psoriasis Group of the Spanish Academy of Dermatology and Venereology. Actas Dermosifiliogr. 2026;117:104544. doi:10.1016/j.ad.2025.104544
Rivera-Díaz R, Daudén E, Carrascosa JM, Cueva P, Puig L. Generalized pustular psoriasis: a review on clinical characteristics, diagnosis, and treatment. Dermatol Ther (Heidelb). 2023;13(3):673-688. doi:10.1007/s13555-022-00881-0
Strober B, Mostaghimi A, Anadkat MJ, et al. Measuring GPPGA, pain, symptom, and quality of life index scores in untreated generalized pustular psoriasis: results from the placebo group of the Effisayil 2 trial. Poster presented at: Winter Clinical Dermatology Conference; January 12–17, 2024; Honolulu, HI.
Armstrong AW, Elston CA, Elewski BE, et al. Generalized pustular psoriasis: a consensus statement from the National Psoriasis Foundation. J Am Acad Dermatol. 2024;90(4):727–730. doi:10.1016/j.jaad.2023.09.080
Gwillim EC, Nichols AJ. Spesolimab for generalized pustular psoriasis: a review of two key clinical trials supporting initial US regulatory approval. Front Immunol. 2024;15:1359481. doi:10.3389/fimmu.2024.1359481