Drug survival in the treatment of generalized pustular psoriasis: A retrospective multicenter study

Kromer, Christian; Loewe, Emilia; Schaarschmidt, Marthe‐Lisa; Pinter, Andreas; Gerdes, Sascha; Herr, Raphael M.; Poortinga, Sietske; Moessner, Rotraut; Wilsmann‐Theis, Dagmar

doi:10.1111/dth.14814

Cited by 39 publications

(61 citation statements)

References 46 publications

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“… 15 The predominance of GPP in adulthood has been demonstrated, and in our sample, the sixth decade of life had the largest number of patients. 4 , 16 , 17 …”

Section: Discussionmentioning

confidence: 99%

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Generalized pustular psoriasis in Brazil: A public claims database study

Duarte¹,

Carvalho

Romiti

et al. 2022

JAAD International

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Background Generalized pustular psoriasis (GPP) is a rare and severe phenotype of psoriasis characterized by sudden outbreak of widespread coalescent sterile pustules associated with a spectrum of systemic symptoms. Objective We aimed to describe the epidemiology and treatment of GPP in Brazil from the public health care system perspective. Methods This was a retrospective public claims database study, using outpatient and inpatient databases, with information from January 2018 to August 2020, based on records of health resource utilization by patients with GPP. Outpatient treatment regimens and fatal inpatient outcomes were described. Results In total, 1458 outpatients of all ages were identified, of whom 53% were women. We estimated the GPP prevalence in Brazil to be between 0.7 and 0.9 per 100,000. Acitretin was the most commonly dispensed drug. Of all the outpatients, 769 outpatients could be tracked in the inpatient database, and 151 had hospital admissions during the study period. Of them, 5.3% had a fatal outcome during hospitalization. A primary skin condition or an infection was the most frequent hospitalization cause. Limitation The International Classification of Diseases codes for GPP and psoriasis have not been previously validated in this context. Conclusion GPP is a rare disease in Brazil and affects individuals of all ages and both sexes. Hospitalizations and disease-related deaths highlight the need for its prompt diagnosis, close medical follow-up, and effective treatment.

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“… 15 The predominance of GPP in adulthood has been demonstrated, and in our sample, the sixth decade of life had the largest number of patients. 4 , 16 , 17 …”

Section: Discussionmentioning

confidence: 99%

“…A study of retrospective data from 5 German university centers showed that 31.4% and 27.3% of treated patients reached partial and no response, respectively, reinforcing the need for satisfactory treatment. 17 …”

Section: Discussionmentioning

confidence: 99%

Generalized pustular psoriasis in Brazil: A public claims database study

Duarte¹,

Carvalho

Romiti

et al. 2022

JAAD International

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“…Extracutaneous manifestations of a GPP flare include arthritis and arthralgia, uveitis and conjunctivitis, and neutrophilic cholangitis [ 3 , 4 , 8 , 35 ]. Generalized pustular psoriasis is also associated with multiple comorbidities, such as hyperlipidemia, hypertension, diabetes mellitus, obesity, anxiety, and depression [ 3 , 32 , 36 ].…”

Section: Clinical Characteristics and Course Of Gppmentioning

confidence: 99%

Clinical Course and Characteristics of Generalized Pustular Psoriasis

2022

Self Cite

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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%.

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“…In a retrospective German multicenter study examining 201 treatment series of 86 GPP patients, biological treatment was found to be significantly more effective than non-biological therapies and the median drug survival was significantly higher with biologicals vs. nonbiologicals. When the drugs were grouped according to the target cytokine, the best retention time was observed for IL-17A inhibitors, followed by IL-(12)/23 inhibitors and TNF-α blockers [ 163 ].…”

Section: Treatmentmentioning

confidence: 99%

Pustular Psoriasis: From Pathophysiology to Treatment

Genovese

Moltrasio

Cassano³

et al. 2021

Biomedicines

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Pustular psoriasis (PP) is a clinicopathological entity encompassing different variants, i.e., acute generalized PP (GPP), PP of pregnancy (impetigo herpetiformis), annular (and circinate) PP, infantile/juvenile PP, palmoplantar PP/palmoplantar pustulosis, and acrodermatitis continua of Hallopeau (ACH), which have in common an eruption of superficial sterile pustules on an erythematous base. Unlike psoriasis vulgaris, in which a key role is played by the adaptive immune system and interleukin (IL)-17/IL-23 axis, PP seems to be characterized by an intense inflammatory response resulting from innate immunity hyperactivation, with prominent involvement of the IL-36 axis. Some nosological aspects of PP are still controversial and debated. Moreover, owing to the rarity and heterogeneity of PP forms, data on prognosis and therapeutic management are limited. Recent progresses in the identification of genetic mutations and immunological mechanisms have promoted a better understanding of PP pathogenesis and might have important consequences on diagnostic refinement and treatment. In this narrative review, current findings in the pathogenesis, classification, clinical features, and therapeutic management of PP are briefly discussed.

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Drug survival in the treatment of generalized pustular psoriasis: A retrospective multicenter study

Cited by 39 publications

References 46 publications

Generalized pustular psoriasis in Brazil: A public claims database study

Generalized pustular psoriasis in Brazil: A public claims database study

Clinical Course and Characteristics of Generalized Pustular Psoriasis

Pustular Psoriasis: From Pathophysiology to Treatment

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