Dataset Information

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

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.

SUBMITTER: Noe MH

PROVIDER: S-EPMC8655659 | biostudies-literature | 2022 Jan

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

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

Noe Megan H MH Wan Marilyn T MT Mostaghimi Arash A Gelfand Joel M JM Agnihothri Ritesh R Armstrong April W AW Bhutani Tina T Bridges Alina A Brownstone Nicholas N Butt Melissa M Duffin Kristina P Callis KPC Carr Christian C Creadore Andrew A DeNiro Katherine L KL Desai Sheena S Dominguez Arturo R AR Duffy Emily K EK Fairley Janet A JA Femia Alisa A Gudjonsson Johann E JE Kaffenberger Jessica A JA Katz Kimberly L KL Le Stephanie T ST Martinez Edgar E Maverakis Emanual E Myers Bridget B Naik Haley B HB Nelson Caroline A CA Ortega-Loayza Alex G AG Plovanich Molly E ME Rangel Lauren K LK Ravi Vignesh V Reddy Vidhatha D VD Saleh Jamal Z JZ Kirby Joslyn S JS Sandhu Jeena K JK Shakshouk Hadir H Shields Bridget E BE Sharif-Sidi Zakariyah Z Smith Jacob J Steahr Amanda A Toussi Atrin A Wanat Karolyn A KA Wang Bo B Wei Brian M BM Weinhammer Annika A Worswick Scott D SD Yang Alexander A

JAMA dermatology 20220101 1

<h4>Importance</h4>Generalized pustular psoriasis (GPP) is a chronic, orphan disease with limited epidemiological data.<h4>Objective</h4>To describe the clinical characteristics, treatments, longitudinal disease course, and disease-specific health care utilization among patients with GPP across the United States.<h4>Design, setting, and participants</h4>A retrospective longitudinal case series involving 95 adults who met the European Rare and Severe Psoriasis Expert Network consensus definition ...[more]

PMID: 34878491

Similar Datasets

Project description:Introduction and importance: Generalized pustular psoriasis of pregnancy (GPPP) is a rare dermatosis that causes maternal and fetal morbidity and mortality. Pustular psoriasis of pregnancy (PPP) is a challenging dermatological Condition, which can have impact on the life pregnant woman's and her unborn child's life. Case presentation Here we report a 26-year-old woman with a history of persistent plaque psoriasis presented with generalized pustular lesions. Ultrasonography revealed normal fetal development despite high serum ESR and CRP levels. Pustular psoriasis was confirmed by histopathology. The patients were given systemic prednisolone 32 mg once daily, which was raised to 60 mg once daily on the tenth day of treatment to manage fresh outbreaks, and the patient's rash continued to gradually improve. When the lesions faded after 4 weeks of treatment, the dose was reduced to 16 mg/day. At 31 weeks' gestation, the patient was discharged and she was kept on prednisolone at a low dose of 4 mg once daily for the duration of the pregnancy. Clinical discussion Generalized pustular psoriasis of pregnancy (GPPP), herpetiformis, is a less common form dermatosis that can be fatal for both mother and the fetus. Response to treatment is good when initiated early in the course of the disease. This present case shows young pregnant mother with GPPP successfully treated with systemic corticosteroid. Conclusion Contrary to the majority of other common pregnant dermatosis, pustular psoriasis is an uncommon condition that can have harmful effects on the fetus. Our patient's PPP symptoms included systemic ones as well as body and palm involvement. Close monitoring and administration of systemic corticosteroids ensured secure outcomes. Highlights • Generalized pustular psoriasis (GPPP) is a rare dermatosis that causes maternal and fetal morbidity and mortality.• This case illustrates a woman that had history of psoriasis vulgaris two years before pregnancy and developed pustular psoriasis type.• Systemic corticosteroids are regarded as the gold standard in the treatment of GPPP.• In this paper, we will present the case report of 26 year old pregnant woman who developed widespread pustular lesions in the 27th week of her fifth pregnancy.

Dataset Information

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

Importance

Objective

Design, setting, and participants

Main outcomes and measures

Results

Conclusions and relevance

Publications

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

Similar Datasets

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