2023-06-26 15:14:00
“Generalized pustular psoriasis (GPP) is a rare, life-threatening, autoinflammatory skin disease characterized by a widespread eruption of sterile pustules over an erythematous base manifesting with or without systemic symptoms such as pain, fever, general malaise, fatigue, and extracutaneous (cholestasis, arthritis), in isolated form or in the presence of a history of plaque psoriasis.In the pathogenesis of Gpp, the dysregulation of the innate immune system prevails, involving in particular the inflammatory pathway of interleukin (IL)-36, with induction of an inflammatory keratinocyte response and neutrophil recruitment”. This was stated by Maria Concetta Fargnoli, vice president of Sidemast – Italian Society of Dermatology, Venereology and Sexually Transmitted Diseases, speaking this morning at the presentation in the Senate of ‘On my skin: living with GPP’, an alliance between associations, experts and institutions to inform and raise awareness of the disease.
“The clinical course of the disease – explains Fargnoli – can be recurrent (with more than one episode) or persistent (for more than 3 months). The frequency of exacerbations varies between patients and exacerbations can be spontaneous or triggered by infections, stressful events medications, drug withdrawal, primarily corticosteroids, electrolyte disturbances, and pregnancy.GPP is associated with significant morbidity and, in some cases, mortality, especially in the absence of appropriate and timely treatment.”
Considered an ultra rare form of psoriasis accounting for less than 1% of all cases of psoriasis, “Gpp is more common in women than men, can occur at any age, but most frequently between the ages of 50 and 60 – underlines the expert – Extreme variability in prevalence has been reported in Western and Asian literature, with values ranging from 1.76 to 124 patients with GPP per million people, reflecting the different methodologies, populations and definitions of GPP used and the inclusion or failure to differentiate between localized and generalized variants of pustular psoriasis. In France, a prevalence of 1.76 patients per million people has been estimated; in Brazil, it is estimated to range between 7 and 9 patients per million; in Korea between 88 and 124 patients per million people per year; 7.46 cases per million in Japan and 1.53 per 100,000 people in Sweden.Data on the overall mortality rate for GPP is relatively limited to studies in specific areas geographic regions, but it is estimated to be between 2% and 7% worldwide. The deaths were attributed to septic shock and cardiorespiratory failure.”
The clinical course of the pathology “is highly variable and in general, without treatment, it is unstable and long-lasting – Fargnoli points out – The important cutaneous involvement, the associated symptoms (itching and pain), the systemic symptoms and the comorbidities contribute to the negative impact on the patient’s quality of life. The relapsing nature of the disease, with recurrent or intermittent exacerbations, and frequent hospitalization exacerbate the significant burden of the disease, and may be accompanied by mental comorbidities such as anxiety and depression and socio-economic comorbidities”.
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