Evaluation of a Case Series of Patients With Palmoplantar Pustulosis in the United States

• Published in: JAMA dermatology (Chicago, Ill.) Vol. 158; no. 1; p. 68
• Main Authors: Noe, Megan H, Wan, Marilyn T, Mostaghimi, Arash, Gelfand, Joel M, Agnihothri, Ritesh, Armstrong, April W, Bhutani, Tina, Bridges, Alina, Brownstone, Nicholas, Butt, Melissa, Duffin, Kristina P Callis, Carr, Christian, Creadore, Andrew, DeNiro, Katherine L, Desai, Sheena, Dominguez, Arturo R, Duffy, Emily K, Fairley, Janet A, Femia, Alisa, Gudjonsson, Johann E, Kaffenberger, Jessica A, Katz, Kimberly L, Kirby, Joslyn S, Le, Stephanie T, Martinez, Edgar, Maverakis, Emanual, Myers, Bridget, Naik, Haley B, Nelson, Caroline A, Ortega-Loayza, Alex G, Plovanich, Molly E, Rangel, Lauren K, Ravi, Vignesh, Reddy, Vidhatha D, Saleh, Jamal Z, Sandhu, Jeena K, Shakshouk, Hadir, Shields, Bridget E, Sharif-Sidi, Zakariyah, Smith, Jacob, Steahr, Amanda, Toussi, Atrin, Wanat, Karolyn A, Wang, Bo, Wei, Brian M, Weinhammer, Annika, Worswick, Scott D, Yang, Alexander
• Format: Journal Article
• Language: English
• Published: United States 01-01-2022
• Subjects: Adult; Chronic Disease; Comorbidity; Female; Humans; Psoriasis - diagnosis; Psoriasis - epidemiology; Psoriasis - therapy; Retrospective Studies; Skin Diseases, Vesiculobullous - epidemiology; United States - epidemiology; United States
• ISSN: 2168-6084
• DOI: 10.1001/jamadermatol.2021.4635

Palmoplantar pustulosis (PPP) is a is a chronic, orphan disease with limited epidemiological data.
To describe the clinical characteristics, treatments, longitudinal disease course, and health care utilization in adults with PPP across the US.
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.
The primary outcome was to describe the patient characteristics, associated medical comorbidities, treatment patterns, complications, and PPP-specific health care utilization.
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.
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.