Faculty Collaboration Database

Evaluation of a Case Series of Patients With Generalized Pustular Psoriasis in the United States. JAMA Dermatol 2022 Jan 01;158(1):73-78

Date

12/09/2021

Pubmed ID

34878491

Pubmed Central ID

PMC8655659

DOI

10.1001/jamadermatol.2021.4640

Scopus ID

2-s2.0-85121237930 (requires institutional sign-in at Scopus site)   23 Citations

Abstract

IMPORTANCE: Generalized pustular psoriasis (GPP) is a chronic, orphan disease with limited epidemiological data.

OBJECTIVE: To describe the clinical characteristics, treatments, longitudinal disease course, and disease-specific health care utilization among patients with GPP across the United States.

DESIGN, SETTING, AND PARTICIPANTS: A retrospective longitudinal case series involving 95 adults who met the European Rare and Severe Psoriasis Expert Network consensus definition for GPP and were treated at 20 US academic dermatology practices between January 1, 2007, and December 31, 2018.

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

RESULTS: Sixty-seven of 95 patients (70.5%) were women (mean age, 50.3 years [SD, 16.1 years]). In the initial encounter, 35 patients (36.8%) were hospitalized and 64 (67.4%) were treated with systemic therapies. In total, more than 20 different systemic therapies were tried. During the follow-up period, 19 patients (35.8%) reported hospitalizations at a median rate of 0.5 hospitalizations per year (IQR, 0.4-1.6). Women had a decreased risk of an emergency department or hospital encounter (odds ratio, 0.19; 95% CI, 0.04-0.83).

CONCLUSIONS AND RELEVANCE: Generalized pustular psoriasis is a rare, chronic disease without standard treatment and is associated with continued health care utilization over time.

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, 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, Kirby JS, 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

Acute Disease
Adult
Chronic Disease
Female
Humans
Middle Aged
Psoriasis
Retrospective Studies
Skin Diseases, Vesiculobullous
United States