Evaluation of a Case Series of Patients with Generalized Pustular Psoriasis in the United States

Megan H. Noe, Marilyn T. Wan, Arash Mostaghimi, Joel M. Gelfand, Ritesh Agnihothri, April W. Armstrong, Tina Bhutani, Alina Bridges, Nicholas Brownstone, Melissa Butt, Kristina P.Callis Duffin, Christian Carr, Andrew Creadore, Katherine L. Deniro, Sheena Desai, Arturo R. Dominguez, Emily K. Duffy, Janet A. Fairley, Alisa Femia, Johann E. GudjonssonJessica A. Kaffenberger, Kimberly L. Katz, Stephanie T. Le, Edgar Martinez, Emanual Maverakis, Bridget Myers, Haley B. Naik, Caroline A. Nelson, Alex G. Ortega-Loayza, Molly E. Plovanich, Lauren K. Rangel, Vignesh Ravi, Vidhatha D. Reddy, Jamal Z. Saleh, Joslyn S. Kirby, Jeena K. Sandhu, Hadir Shakshouk, Bridget E. Shields, Zakariyah Sharif-Sidi, Jacob Smith, Amanda Steahr, Atrin Toussi, Karolyn A. Wanat, Bo Wang, Brian M. Wei, Annika Weinhammer, Scott D. Worswick, Alexander Yang

Research output: Contribution to journalArticlepeer-review

19 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)73-78
Number of pages6
JournalJAMA Dermatology
Issue number1
StatePublished - Jan 2022

All Science Journal Classification (ASJC) codes

  • Dermatology


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