The Chronicity and Disease Burden of Generalized Pustular Psoriasis

Abstract

Introduction: Generalized pustular psoriasis (GPP) is a rare, inflammatory skin disease characterized by sudden, widespread eruption (flare) of small sterile pustules often with systemic symptoms. Many patients experience chronic symptoms between flares and may require continuous management.

Methods: We describe the long-term burden of GPP and its effects on quality of life (QoL) in 10 patients who presented to US clinical practices with GPP flare.

Results: Patients were aged 18–92 years with comorbidities (e.g., hypertension, plaque psoriasis, obesity, type 2 diabetes, hypercholesterolemia, diverticulitis, dementia, depression). At presentation, all patients had visible signs of GPP, plus pain, itch, or a sensation of heat in the skin; several experienced systemic symptoms, including leukemoid reaction, painful joints/muscles, fever, or altered mental status. Several patients presented to the emergency room; over half required hospitalization for management. Reported flare triggers included hydroxychloroquine treatment, corticosteroid withdrawal, stress, and COVID-19 infection, though for some, the trigger was unknown. All patients previously received multiple off-label medications for their GPP symptoms. GPP flare was initially misdiagnosed in 6 patients: acute generalized exanthematous pustulosis (2), guttate psoriasis, nummular dermatitis, rheumatoid arthritis and lupus, and contact dermatitis. All misdiagnosed cases received oral or topical corticosteroid treatment, which failed or worsened GPP flare and delayed initiation of targeted GPP treatment. Diagnosis of GPP was usually achieved via skin biopsy. All patients experienced chronic GPP symptoms requiring long-term management. Patients reported a continuous impact of GPP on QoL. One patient’s activities of daily living were severely affected, another reported joint and muscle pain prevented her from walking, and an 18-year-old patient reported being bullied because of his skin’s appearance. For 6 patients this was their first flare, while the others experienced flare recurrence. One patient had 1 previous flare and 3 patients had experienced multiple flares. One of these patients, now aged 62 years, had experienced numerous flares since childhood. GPP flares led to severe complications and morbidity in several patients, including multiple organ failure, fever, and altered mental status during the flare.

 Conclusions: GPP is associated with both recurrent flares and chronic symptoms between flares, leading to decreased patient QoL, increased morbidity, and risk of complications. Thus, safe and effective long-term treatment options for GPP are needed to mitigate disease burden.