IMPORTANCEAcute generalized exanthematous pustulosis (AGEP) is a rare, severe cutaneous adverse reaction associated with systemic complications. Currently available data are largely limited to small retrospective case series.OBJECTIVE To describe the clinical characteristics, disease course, and outcomes of a heterogeneous group of patients with AGEP across the US. DESIGN, SETTING, AND PARTICIPANTSA retrospective review of a case series of patients was conducted from January 1, 2000, through July 31, 2020. All 340 included cases throughout 10 academic health systems in the US were scored retrospectively using the EuroSCAR scoring system, and patients with a score corresponding to probable or definite AGEP and aged 18 years or older were included. MAIN OUTCOMES AND MEASURESPatient demographic characteristics, clinical course, suspected causative agent, treatment, and short-and long-term outcomes.RESULTS Most of the 340 included patients were women (214 [62.9%]), White (206 [60.6%]), and non-Hispanic (239 [70.3%]); mean (SD) age was 57.8 (17.4) years. A total of 154 of 310 patients (49.7%) had a temperature greater than or equal to 38.0 °C that lasted for a median of 2 (IQR, 1-4) days. Of 309 patients, 263 (85.1%) developed absolute neutrophilia and 161 patients (52.1%) developed either absolute or relative eosinophilia. Suspected causes of AGEP were medications (291 [85.6%]), intravenous contrast agents (7 [2.1%]), infection (3 [0.9%]), or unknown (39 [11.5%]). In 151 cases in which a single medication was identified, 63 (41.7%) were β-lactam antimicrobials, 51 (33.8%) were non-β-lactam antimicrobials, 9 (6.0%) were anticonvulsants, and 5 (3.3%) were calcium channel blockers. The median time from medication initiation to AGEP start date was 3 (IQR, 1-9) days. Twenty-five of 298 patients (8.4%) had an acute elevation of aspartate aminotransferase and alanine aminotransferase levels, with a peak at 6 (IQR, 3-9) days. Twenty-five of 319 patients (7.8%) experienced acute kidney insufficiency, with the median time to peak creatinine level being 4 (IQR, 2-5) days after the AGEP start date. Treatments included topical corticosteroids (277 [81.5%], either alone or in combination), systemic corticosteroids (109 [32.1%]), cyclosporine (10 [2.9%]), or supportive care only (36 [10.6%]). All-cause mortality within 30 days was 3.5% (n = 12), none of which was suspected to be due to AGEP.CONCLUSIONS AND RELEVANCE This retrospective case series evaluation of 340 patients, the largest known study cohort to date, suggests that AGEP onset is acute, is usually triggered by recent exposure to an antimicrobial, may be associated with liver or kidney complications in a minority of patients, and that discontinuation of the triggering treatment may lead to improvement or resolution.

almoplantar pustulosis (PPP) is a limited form of pustular psoriasis characterized by persistent, sterile macroscopic pustules on the palms and/or soles and presents both in isolation and in patients with a history of plaque psoriasis. Previous research suggests there is a genetic overlap between PPP and plaque psoriasis, 1-4 but despite any similarities, there are also key differences. Palmoplantar pustulosis predominantly presents in women, while psoriasis occurs in men and women equally. Despite the limited body surface area associated with PPP compared with psoriasis, patients with palmoplantar psoriasis have been shown to experience greater health-related quality-of-life impairment than those with moderate to severe plaque psoriasis. 5,6 Finally, treatments for PPP and palmoplantar psoriasis are similar, but much less is known about the response to treatment in patients with PPP. The objective of this study is to describe the clinical characteristics, longitudinal disease course, treatments, and health care utilization in patients with PPP across the US. Methods Study Design and PopulationThis is a retrospective case series of adults (age ≥18 years) with a diagnosis of PPP made by a dermatologist between January 1, 2007, and December 31, 2018. Up to 10 potential cases were identified from each of 20 sites using electronic health records and/or site-specific databases, starting with cases seen most recently. All diagnoses were confirmed by the principal investigator at each site at the time of data entry, and only patients who met the European Rare and Severe Psoriasis Expert Network consensus definition for PPP-documentation of primary, persistent (<3 months), sterile, macroscopic pustules on the palms and/or soles, not occurring within psoriatic plaques 7 -and had a dermatology encounter with active pustular disease during the study period were included. This study was granted exempt status by the University of Penn-IMPORTANCE Palmoplantar pustulosis (PPP) is a is a chronic, orphan disease with limited epidemiological data. OBJECTIVE To describe the clinical characteristics, treatments, longitudinal disease course, and health care utilization in adults with PPP across the US.

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