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Evaluation of a Case Series of Patients With Palmoplantar Pustulosis in the United States. JAMA Dermatol 2022 Jan 01;158(1):68-72

Date

12/09/2021

Pubmed ID

34878495

Pubmed Central ID

PMC8655660

DOI

10.1001/jamadermatol.2021.4635

Scopus ID

2-s2.0-85121229692 (requires institutional sign-in at Scopus site)   15 Citations

Abstract

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.

DESIGN, SETTING, AND PARTICIPANTS: This retrospective, longitudinal case series from 20 academic dermatology practices in the US included a consecutive sample of 197 adults who met the European Rare and Severe Psoriasis Expert Network consensus definition for PPP between January 1, 2007, and December 31, 2018. Data analysis was performed June 2020 to December 2020.

MAIN OUTCOMES AND MEASURES: The primary outcome was to describe the patient characteristics, associated medical comorbidities, treatment patterns, complications, and PPP-specific health care utilization.

RESULTS: Of 197 patients, 145 (73.6%) were female, and the mean (SD) age at presentation was 53.0 (12.6) years, with a mean (SD) follow-up time of 22.1 (28.0) months. On initial presentation, 95 (48.2%) patients reported skin pain, and 39 (19.8%) reported difficulty using hands and/or feet. Seventy patients (35.5%) were treated with systemic treatments, and use of more than 20 different systemic therapies was reported. In patients with at least 6 months of follow-up (n = 128), a median (IQR) of 3.7 (4-10) dermatology visits per year were reported; 24 (18.8%) patients had 5 or more visits during the study period.

CONCLUSIONS AND RELEVANCE: In this case series, PPP was associated with persistent symptoms, continued health care utilization, and a lack of consensus regarding effective treatments, emphasizing the unmet medical need in this population. Additional research is necessary to understand treatment response in these patients.

Author List

Noe MH, Wan MT, Mostaghimi A, Gelfand JM, Pustular Psoriasis in the US Research Group, Agnihothri R, Armstrong AW, Bhutani T, Bridges A, Brownstone N, Butt M, Duffin KPC, Carr C, Creadore A, DeNiro KL, Desai S, Dominguez AR, Duffy EK, Fairley JA, Femia A, Gudjonsson JE, Kaffenberger JA, Katz KL, Kirby JS, Le ST, Martinez E, Maverakis E, Myers B, Naik HB, Nelson CA, Ortega-Loayza AG, Plovanich ME, Rangel LK, Ravi V, Reddy VD, Saleh JZ, Sandhu JK, Shakshouk H, Shields BE, Sharif-Sidi Z, Smith J, Steahr A, Toussi A, Wanat KA, Wang B, Wei BM, Weinhammer A, Worswick SD, Yang A

Author

Karolyn A. Wanat MD Chair, Professor in the Dermatology department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Adult
Chronic Disease
Comorbidity
Female
Humans
Psoriasis
Retrospective Studies
Skin Diseases, Vesiculobullous
United States