A stricter criterion for the classification of patients with generalized pustular psoriasis (GPP) elevates the mortality rate for this group of patients, according to a study published in the Journal of the American Academy of Dermatology.
GPP is a severe form of pustular psoriasis that is driven by inflammation and is associated with several comorbidities, most prominently psoriasis vulgaris. The severity of this rare disorder and the tendency for patients to deteriorate rapidly over time causes mortality figures for patients with this condition to be consistently high.
The authors of the study sought to better understand the mortality rate among this population of patients. They accessed the Swedish National Patient Register and identified 1022 diagnosed cases of GPP; these cases were either primary or secondary. The research team then recruited matched controls based on sex and age. They then identified age-stratified hazard ratios for all-cause mortality via statistical studies.
In a subgroup analysis, the research team limited the definition of GPP to patients with 2 or more GPP diagnoses given at different times during the study period. This represented a stricter criterion for the classification of GPP; around 44% of the patients analyzed met this stricter criterion for GPP diagnosis.
Using this criterion, the research team found that patients with GPP had around 50% higher mortality rate compared with the control. The trajectory of disease progression tends to begin shortly after diagnosis, during which patients experience life-threatening complications triggered by external factors such as drugs, infection, or hypocalcemia. After this initial acute episode, the survival rate for patients with GPP steadily decreases and is consistently lower than the control. The researchers found that the leading cause of death among patients with GPP was circulatory systemic disease, followed by respiratory and digestive diseases.
In conclusion, the authors demonstrated that patients who were diagnosed with the stricter criterion for GPP had higher mortality rates and hazard ratios for all-cause mortality compared with individuals without this condition or those who had psoriasis vulgaris only.
References:
Ericsson O, Löfvendahl S, Norlin JM, Gyllensvärd H, Schmitt-Egenolf M. Mortality in generalized pustular psoriasis: a population-based national register study. J Am Acad Dermatol. Published online May 23, 2023. doi:10.1016/j.jaad.2023.04.066
