Clinical Course and Characteristics of Generalized Pustular Psoriasis

Abstract

Generalized pustular psoriasis (GPP) is a rare, potentially life-threatening disease characterized by episodes of widespread sterile macroscopic pustules, with or without systemic inflammation and/or plaque psoriasis. Multiple GPP subtypes have been described, from acute GPP of von Zumbusch to milder, annular pustular psoriasis. Generalized pustular psoriasis mainly affects adults, with a female preponderance, but juvenile GPP also occurs. Flares are a hallmark of GPP and may occur de novo or be provoked by triggers, including withdrawal of systemic corticosteroids, infections, stress, pregnancy, and menstruation. Severity of flares varies widely between patients, and between flares in an individual patient. Significant flares are often accompanied by systemic symptoms, notably fever, general malaise, and extracutaneous manifestations such as arthritis, uveitis, and neutrophilic cholangitis. Common laboratory abnormalities include neutrophilia, elevated C-reactive protein levels, hypocalcemia, and abnormal liver function tests. The clinical course of GPP is highly variable; it can be a relapsing disease with recurrent flares and no pustulation between flares or a persistent disease with perpetual mild pustulation punctuated with flares of greater severity. Patients may have multiple flares per year or a flare every few years. Most flares last 2–5 weeks and approximately 50% require hospitalization. Life-threatening complications include sepsis and renal, hepatic, respiratory, and heart failure. Reported mortality rates are 2–16%.

Spesolimab: First Approval

“…Generalized pustular psoriasis (GPP) is a rare and potentially-life threatening disease characterized by extensive eruption of pustules on the skin, with or without systemic inflammation and with or without plaque psoriasis [ 1 – 3 ]. GPP flares may occur de novo or in response to triggers such as infection, stress, pregnancy and discontinuation of corticosteroids [ 1 , 2 ]. While the frequency and severity of flares is variable between patients, most flares last between 2 and 5 weeks and around half require hospitalization [ 1 ].…”

“…GPP flares may occur de novo or in response to triggers such as infection, stress, pregnancy and discontinuation of corticosteroids [ 1 , 2 ]. While the frequency and severity of flares is variable between patients, most flares last between 2 and 5 weeks and around half require hospitalization [ 1 ]. Complications can be life-threatening, and include sepsis, kidney failure, liver failure and heart failure [ 1 ].…”

De novo annular pustular psoriasis following mRNA COVID‐19 vaccine

“…APP is a rare variant of generalized pustular psoriasis (GPP) in which the plaques are distributed in a circinate pattern with scaling and pustules on the edges of the lesions, with mild-to-no systemic symptoms. 2 It could be induced by drugs, steroid withdrawal, infections and, rarely, vaccinations. 3 Recently, cases of GPP related to COVID-19 vaccines have been described.…”

Severe Generalized Pustular Psoriasis Successfully Treated with Ixekizumab: A Case Report

“…Two types exist: one is associated with psoriasis vulgaris, while in the other GPP occurs as the sole phenotype. GPP can be triggered by infections, pregnancy, or drugs and is characterized by a sudden eruption of nonbacterial neutrophilic pustules that can cover the entire body surface [ 2 ]. Usually, it is accompanied by fever, leukocytosis, and high level of C-reactive protein.…”