No abstract

No abstract

No abstract

eneralized pustular psoriasis (GPP) is an orphan disease characterized by the rapid appearance of sterile pustules and generalized erythema. Patients are often systemically ill and may experience severe organ dysfunction and rarely death. The genetic risk factors for pustular psoriasis are different from other types of psoriasis. To date, variations have been identified in the following genes: IL36RN (interleukin-36 receptor antagonist), CARD14 (caspase recruitment domain family member 14), AP1S3 (adapter related protein complex 1 subunit sigma 3), SERPINA3 (serpin family A member 3), and MPO (myeloperoxidase); however, the majority of patients do not have a known genetic variant. 1 Owing to the rarity of GPP, there is limited information about the natural disease course. The only epidemiological data from the United States is a report of 63 patients seen over 29 years at a single institution. 2 The objective of this study is to describe the clinical characteristics, natural disease course, treatments, and health care utilization of patients with GPP across the United States. Methods Study Design and PopulationThis is a retrospective, longitudinal case series of adults (≥18 years) with a diagnosis of GPP confirmed by a dermatologist (January 1, 2007-December 31, 2018) (Table 1). Up to 5 potential cases were identified from each of 20 participating sites' electronic health records 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. Only patients who met the European Rare and Severe Psoriasis Expert Network (ERASPEN) consensus definition of GPP with documentation of "primary, sterile, macroscopically visible pustules on nonacral skin, excluding cases where pustulation is restricted to psoriatic plaques" 3 and had had a dermatology encounter with active pustular disease during the study period were included.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 PARTICIPANTSA 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 MEASURESThe 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%) rep...

The SADL model uses simple, readily accessible clinical variables to predict all-cause mortality in Scl-ILD.

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.

Background Supportive care is the cornerstone of management of adult and paediatric Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). However, consensus on the modalities of supportive care is lacking. Objectives Our aim in this international multicentric Delphi exercise was to establish a multidisciplinary expert consensus to standardize recommendations regarding supportive care in the acute phase of SJS/TEN. Methods Participants were sent a survey via the online tool SurveyMonkey, consisting of 103 statements organized into 11 topics: multidisciplinary team composition, suspect drug management, infection prevention, fluid resuscitation and prevention of hypothermia, nutritional support, pain and psychological distress management, management of acute respiratory failure, local skincare, ophthalmological management, management of other mucosa, and additional measures. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). The results were analysed according to the RAND/UCLA Appropriateness Method. Results Forty-five participants from 13 countries (on three continents) participated. After the first round, a consensus was obtained for 82.5% of the 103 initially proposed statements. After the second round, a final consensus was obtained for 102 statements. Conclusions We have reached an international Delphi-based consensus on best supportive care practice for SJS/TEN. Our expert consensus should help guide physicians in treating patients with SJS/TEN and thereby improve short-term prognosis and the risk of sequelae.What is already known about this topic?• Supportive care is the cornerstone of management of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in the acute phase.• There is no consensus or guideline on the best supportive care.

Psoriasis is a chronic inflammatory condition characterized by systemic immune dysregulation. Over the past several years, advances in genetics, microbiology, immunology, and mouse models have revealed the complex interplay between the heritable and microenvironmental factors that drive the development of psoriatic inflammation. In the first of this two-part review series, the authors will discuss the newest insights into the pathogenesis of psoriatic disease and highlight how the evolution of these scientific fields has paved the way for a more personalized approach to psoriatic disease treatment.