Clinical Characteristics and Outcomes of Generalized Pustular Psoriasis Flares

Abstract

Background: Generalized pustular psoriasis (GPP) is a rare, neutrophilic skin disease that can become life-threatening if flares are untreated. There are limited data describing the characteristics and clinical course of GPP disease flares with current treatment options. Objective: To describe the characteristics and outcomes of GPP flares using historical medical information from patients enrolled in the Effisayil™ 1 trial. Methods: Investigators collected retrospective medical data characterizing patients’ GPP flares prior to clinical trial enrollment. Data on overall historical flares were collected, as well as information on patients’ typical, most severe, and longest past flares. This included data on systemic symptoms, flare duration, treatment, hospitalization, and time to clearance of skin lesions. Results: In this cohort (N=53), patients with GPP experienced a mean of 3.4 flares per year. Flares were painful, associated with systemic symptoms, and often triggered by stress, infections, or treatment withdrawal. Resolution of flares was longer than 3 weeks in 57.1%, 71.0%, and 85.7% of documented (or identified) typical, most severe, and longest flares, respectively. GPP flares led to patient hospitalization in 35.1%, 74.2%, and 64.3% of patients for their typical, most severe, and longest flares, respectively. For the majority of patients, pustules took up to 2 weeks to clear for a typical flare and 3–8 weeks to clear for the most severe and longest flares. Conclusion: Our findings highlight that current treatment options are slow to control GPP flares and provide context for assessing the efficacy of new therapeutic strategies in patients with a GPP flare.

Acquired diffuse palmoplantar erythema with keratoderma in Chinese patients with pustular psoriasis: A predictor for IL36 receptor antagonist c.115+6T>C mutation?

“…There are significant diversity in the clinical presentation and genetic background of GPP 15 . Previous studies have identified certain genotype–phenotype correlations among DITRA patients regarding disease manifestation and treatment response.…”

“…There are significant diversity in the clinical presentation and genetic background of GPP. 15 disease manifestation and treatment response. Null IL36RN mutations were associated with GPP, while patients with hypomorphic IL36RN mutations were more likely to develop localized forms of pustular psoriasis.…”

International Consensus Definition and Diagnostic Criteria for Generalized Pustular Psoriasis From the International Psoriasis Council

“…Therefore, diagnosis should not be delayed by any predefined duration of pustulation or BSA. 2,3,17 Distribution on different anatomical sites and multiple erythematous patches with pustules were considered more important clues to diagnosing GPP. There was a 100% consensus that pustular lesions on acral regions may be present during flares and should not rule out a diagnosis of GPP.…”

A case of generalized pustular psoriasis with discrepant resolution time between pustules and erythema after treatment with spesolimab

“…In general, pustules disappear faster than erythema when GPP is treated, including with topical, systemic, and biologic treatments. 2,3 The cytokine balance F I G U R E 1 (a) Histopathological findings for a neutrophil-filled pustule under the stratum corneum and inflammatory cell infiltrates consisting mainly of lymphocytes and neutrophils around capillaries in the papillary dermis layer (hematoxylin and eosin staining: ×100 and ×400). (b) The erythema and pustules on the lower abdomen improved after treatment.…”