Impact of Generalized Pustular Psoriasis from the Perspective of People Living with the Condition: Results of an Online Survey

Abstract

Background Generalized pustular psoriasis (GPP) is a rare disease characterized by episodic worsening (flares). Knowledge of the burden of GPP and the experience of affected individuals is limited. Aims To conduct a survey of people living with GPP to understand how they experience GPP flares, which therapies they have received and are receiving, and how GPP impacts their activities of daily living. Methods The online survey consisted of 43 questions answered by individuals recruited from an opt-in market research database. The research team performed a targeted outreach to identify individuals with GPP. The survey included screening questions to determine if potential participants qualified for inclusion. Eligible individuals were US residents aged ≥ 18 years who self-reported that they had been diagnosed with GPP. Respondents provided consent to participate and received compensation (fair market value) for their time. Results Between August 4 and 14, 2020, 66 people living with GPP in the USA were surveyed. Most participants were female, aged 40-59 years, had been diagnosed ≥ 1 year previously, and had experienced ≥ 2 flares in the past year. A substantial proportion of respondents had symptoms for years, had consulted multiple healthcare professionals, and experienced misdiagnoses before receiving a diagnosis of GPP. Emotional stress was the most common cause of flares and many respondents reported a fear of flares. Respondents defined flares by the presence of itching, an increase in the size of the affected area, more crusts or pustules, and fatigue. A change in mood was the most burdensome symptom. Most respondents were receiving topical corticosteroids and only approximately one-third felt their condition was well controlled. GPP had an impact on activities of daily living even in the absence of flares and many respondents felt that their physician did not understand the level of emotional, psychological, or physical pain caused by GPP. Conclusions GPP imposes a substantial emotional burden on patients, with wide-ranging impacts on activities of daily living beyond the physical discomfort of skin lesions.

An Overview of Clinical Manifestations of Dermatological Disorders in Intensive Care Units: What Should Intensivists Be Aware of?

“…The incidence is highest between the ages of 40 and 59. It is distinguished by the widespread eruption of sterile pustules, which may be accompanied by generalized symptoms of pain, fever, malaise, or fatigue [ 75 , 76 ].…”

Generalized pustular psoriasis: the case for rare disease and orphan designation

“…Dear Editor, Generalized pustular psoriasis (GPP) is a life‐threatening rare disease that has a significant impact on patients’ daily activities 1–4 . Owing to a lack of awareness of GPP among doctors, this disease is unsatisfactorily diagnosed and inadequately treated 1 . Evidence for the efficacy of current medications is lacking, and no treatments are approved for GPP in Europe or the USA 2–4 .…”

“…We would like to thank Lars Werner (Psoriasisforeningen, Copenhagen, Denmark) who also participated as a PAG representative in all roundtable meetings and questionnaires. Jan Koren, 1 Sicily Mburu, 2 David Trigos, 1 Giovanni Damiani 3,4,5 and Luigi Naldi iD 6…”

“…[1][2][3][4] Owing to a lack of awareness of GPP among doctors, this disease is unsatisfactorily diagnosed and inadequately treated. 1 Evidence for the efficacy of current medications is lacking, and no treatments are approved for GPP in Europe or the USA. [2][3][4] Despite the rarity of GPP, it remains to be conclusively stated whether this disease fulfils all the criteria for European Medicines Agency (EMA) orphan designation.…”

Foreword

“…Finally, Dale R. Reisner, Frida Dunger Johnsson, Nirali Kotowsky, Steven Brunette, Wendell Valdecantos, and Kilian Eyerich report on the outcomes of a survey on the burden of GPP in people with the condition, and highlight the substantial emotional burden of GPP and its impact on daily life of patients [ 7 ].…”