Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening conditions with high morbidity and mortality. Supportive care management of SJS/TEN is highly variable. A systematic review of the literature was performed by dermatologists, ophthalmologists, intensivists and gynecologists with expertise in SJS/TEN to generate statements for supportive care guideline development. Members of the Society of Dermatology Hospitalists (SDH) with expertise in SJS/TEN were invited to participate in a modified, online Delphi-consensus. 9-point Likert scale questionnaires regarding 135 statements were administered. The RAND/UCLA appropriateness method was employed to evaluate and select proposed statements for guideline inclusion; statements with median ratings of 6.5-9 and disagreement index ≤1 were included in the guideline. For the final round, the guidelines were appraised by all the participants. An evidence-based discussion and recommendations for hospital setting and care team, wound care, ocular care, oral care, urogenital care, pain management, infection surveillance, fluid and electrolyte management, nutrition and stress ulcer prophylaxis, airway management, and anticoagulation in adult patients with SJS/TEN are included.
almoplantar pustulosis (PPP) is a limited form of pustular psoriasis characterized by persistent, sterile macroscopic pustules on the palms and/or soles and presents both in isolation and in patients with a history of plaque psoriasis. Previous research suggests there is a genetic overlap between PPP and plaque psoriasis, 1-4 but despite any similarities, there are also key differences. Palmoplantar pustulosis predominantly presents in women, while psoriasis occurs in men and women equally. Despite the limited body surface area associated with PPP compared with psoriasis, patients with palmoplantar psoriasis have been shown to experience greater health-related quality-of-life impairment than those with moderate to severe plaque psoriasis. 5,6 Finally, treatments for PPP and palmoplantar psoriasis are similar, but much less is known about the response to treatment in patients with PPP. The objective of this study is to describe the clinical characteristics, longitudinal disease course, treatments, and health care utilization in patients with PPP across the US.
Methods
Study Design and PopulationThis is a retrospective case series of adults (age ≥18 years) with a diagnosis of PPP made by a dermatologist between January 1, 2007, and December 31, 2018. Up to 10 potential cases were identified from each of 20 sites using electronic health records and/or site-specific databases, starting with cases seen most recently. All diagnoses were confirmed by the principal investigator at each site at the time of data entry, and only patients who met the European Rare and Severe Psoriasis Expert Network consensus definition for PPP-documentation of primary, persistent (<3 months), sterile, macroscopic pustules on the palms and/or soles, not occurring within psoriatic plaques 7 -and had a dermatology encounter with active pustular disease during the study period were included. This study was granted exempt status by the University of Penn-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.
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