Study Finds No Standardized Treatment, Continued Health Care Utilization in Pustular Psoriasis

A case series of patients with pustular psoriasis indicated a lack of standardized treatment and continued health care utilization, in which men were at greater risk of an emergency department or hospital encounter.

There remains no standardized treatment for generalized pustular psoriasis (GPP), with continued health care utilization experienced by these patient populations, according to study findings published recently in JAMA Dermatology.

As an orphan disease characterized by the rapid appearance of sterile pustules and generalized erythema, GPP has been indicated to have genetic risk factors different from other types of psoriasis. Moreover, the researchers note that patients with the rare, chronic disease are often systemically ill and may experience severe organ dysfunction, and rarely death.

With limited information on the pathophysiology of GPP, they conducted a retrospective longitudinal case series to examine the clinical characteristics, natural disease course, treatments, and health care utilization of affected US patients.

The study included 95 adult patients with GPP (mean [SD] age, 50.3 [16.1] years; 70.5% female) who met the European Rare and Severe Psoriasis Expert Network consensus definition for the disease and were treated at 20 US academic dermatology practices between January 1, 2007, and December 31, 2018.

Regarding initial presentation of disease, 35 patients (36.8%) presented during an inpatient admission, 9 (9.5%) in the emergency department (ED), and 51 (53.7%) during an outpatient or ambulatory dermatology office visit. Of the 57 patients who had no prior history of GPP, 29 (50%) were inpatient admissions or ED encounters.

The presence of pustules on the trunk and extremities was reported in a majority of patients, with several other symptoms, as well as comorbidities, associated with GPP:

  • Skin pain (62.1%; n = 59)
  • Joint pain (26.2%; n = 25)
  • Tachycardia (16.8%; n = 16)

The most common comorbidities identified were hypertension, depression, diabetes, chronic kidney disease, and hypothyroidism

Furthermore, during the initial encounter, most patients were given systemic therapies (n = 64), in which more than 20 systemic therapies were tried. Health care utilization was examined in 53 patients with at least 6 months of follow-up, with 19 patients (35.8%) reporting hospitalization for GPP symptoms at a median (interquartile range) rate of 0.5 (0.4-1.6) hospitalizations per year.

The median number of dermatology visits reported was 3.2 (2.2-6.1) per year, with a maximum of 18 visits per year of follow-up time. Women were shown to be at a 81% decreased risk of an ED encounter or hospital admission during follow-up in age- and sex-adjusted models (OR, 0.19; 95% CI, 0.04-0.83).

“The results of this case series evaluation support prior findings that GPP is a rare, chronic disease without standard treatment and is associated with continued health care utilization over time,” concluded the researchers. “Further prospective research is necessary to better understand treatment efficacy in patients with GPP.”


Noe MH, Wan MT, Mostaghimi A, et al. Evaluation of a case series of patients with generalized pustular psoriasis in the United States. JAMA Dermatol. Published online December 8, 2021. doi:10.1001/jamadermatol.2021.4640

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