Generalized Pustular Psoriasis: A Review on Clinical Characteristics, Diagnosis, and Treatment.

Generalized pustular psoriasis (GPP) is a rare, chronic, and severe inflammatory skin disorder characterized by sudden eruption of sterile pustules, often accompanied by systemic inflammation. GPP flares can be life-threatening if untreated, owing to potential serious complications such as sepsis and cardiovascular failure. Diagnosis and clinical measurement of disease severity in GPP are often difficult. Lack of standardized criteria in the international guidelines and the heterogeneity of cutaneous and extracutaneous symptoms make the diagnosis of GPP difficult. Clinical criteria for description and diagnosis of pustular conditions, including GPP, are variable and there is no specific agreement on commonly sustained concepts. Differentiation of GPP from other similar conditions/diseases is important and requires careful assessments. The evidence that supports current topical or systemic therapies is largely based on case reports and small studies. Some biologic agents that target key cytokines involved in the activation of inflammatory pathways have been used as treatments for GPP. Recently, spesolimab, an IL-36R antagonist, has been approved in the USA and Japan for the treatment of GPP flares in adults, but there are no currently approved treatments for GPP in Europe. The IL-36 pathway has recently emerged as a central axis driving the pathogenic inflammatory mechanisms of GPP. Biologic agents that inhibit the IL-36 pathway have shown efficacy and safety in patients with GPP, addressing a generally considered unmet medical need.

Efficacy of dimethyl fumarate treatment for moderate-to-severe plaque psoriasis: presentation extracts from the 29th EADV virtual congress, 29-31 October 2020.

Background: The 29th EADV Virtual Congress took place between the 29th-31st of October 2020. On October 29th, there was a Session on systemic treatment in which Professors Ulrich Mrowietz and Mar Llamas-Velasco presented the latest research on the efficacy of dimethyl fumarate (DMF) treatment for moderate-to-severe plaque psoriasis (BRIDGE and DIMESKIN 1 studies, respectively). The accepted DMF abstract from Professor Matthias Augustin, on the SKILL study, is also presented here. Results: Data from either prospective interventional (BRIDGE) or non-interventional (DIMESKIN 1, SKILL) studies among patients with moderate-to-severe psoriasis showed that DMF provides a positive efficacy profile in all four body regions included in the Psoriasis Area and Severity Index assessment (head and neck, trunk, upper and lower extremities) and a particularly interesting profile (strong efficacy) in the head and neck region. These findings may be of special interest to patients with scalp psoriasis who have been using topical therapies for a long time. Patient-reported outcomes (quality of life, pruritus) also improved during the 24 weeks of DMF treatment. The safety profile of DMF was similar to the previously described with fumaric acid esters. Conclusions: In summary, these results confirm the favorable efficacy and safety profile of DMF in long-term treatment.

Estado actual de la atención multidisciplinar para pacientes con artritis psoriásica en España: proyecto NEXUS 2.0 / Current status of multidisciplinary care in psoriatic arthritis in Spain: NEXUS 2.0 project

OBJETIVO: 1) Analizar la implementación de los modelos de atención multidisciplinar en pacientes con artritis psoriásica (APs), y 2) definir estándares de calidad de mínimos y de excelencia. MÉTODOS: Se envió una encuesta a profesionales que ya realizan atención multidisciplinar o están en vías preguntando por: 1) tipo de modelo de abordaje multidisciplinar, y 2) grado, prioridad y facilidad de la implementación de los estándares de calidad de estructura, proceso y resultado. En 6 reuniones regionales se presentaron y discutieron los resultados de la encuesta, tanto a nivel nacional como regional, y se definió la prioridad definitiva de los estándares de calidad. En una reunión de grupo nominal, 11 expertos (reumatólogos y dermatólogos) analizaron los resultados de la encuesta y las reuniones regionales. Con ello definieron qué estándares de calidad son actualmente de mínimos y cuáles de excelencia. RESULTADOS: Los modelos de atención multidisciplinar conjunto y paralelo son los más implementados, y los de los estándares de calidad es muy variable: en los de estructura varía del 22 al 74%, en los de proceso del 17 al 54% y en los de resultado del 2 al 28%. De los 25 estándares de calidad originales, 9 se consideran solo de mínimos, 4 de excelencia y 12 tienen definidos unos criterios para ser de mínimos y otros para la excelencia. CONCLUSIONES: La definición de estándares de calidad de mínimos y de excelencia ayudará en la consecución del objetivo de la atención multidisciplinar para pacientes con APs, que es la mejor asistencia sanitaria posible

OBJECTIVE: 1) To analyze the implementation of multidisciplinary care models in psoriatic arthritis (PsA) patients, 2) To define minimum and excellent standards of care. METHODS: A survey was sent to clinicians who already performed multidisciplinary care or were in the process of undertaking it, asking: 1) Type of multidisciplinary care model implemented; 2) Degree, priority and feasibility of the implementation of quality standards in the structure, process and result for care. In 6 regional meetings the results of the survey were presented and discussed, and the ultimate priority of quality standards for care was defined. At a nominal meeting group, 11 experts (rheumatologists and dermatologists) analyzed the results of the survey and the regional meetings. With this information, they defined which standards of care are currently considered as minimum and which are excellent. RESULTS: The simultaneous and parallel models of multidisciplinary care are those most widely implemented, but the implementation of quality standards is highly variable. In terms of structure it ranges from 22% to 74%, in those related to process from 17% to 54% and in the results from 2% to 28%. Of the 25 original quality standards for care, 9 were considered only minimum, 4 were excellent and 12 defined criteria for minimum level and others for excellence. CONCLUSIONS: The definition of minimum and excellent quality standards for care will help achieve the goal of multidisciplinary care for patients with PAs, which is the best healthcare possible

Modelos de atención multidisciplinar en pacientes con artritis psoriásica en España / Multidisciplinary care models for patients with psoriatic arthritis

Objetivo. Describir la estructura y procesos de distintos modelos de atención multidisciplinar de pacientes con artritis psoriásica (APs) en España, así como las barreras y facilitadores en su implantación. Métodos. Se realizó un estudio cualitativo mediante entrevistas estructuradas a 24 profesionales (12 reumatólogos y 12 dermatólogos que realizan atención multidisciplinar en pacientes con APs). Se recogieron datos relacionados con el centro, servicio, población atendida y sobre el modelo de atención multidisciplinar (tipo, recursos materiales y humanos, requerimientos de los profesionales, objetivos, criterios de entrada y salida, agendas, protocolos de actuación, responsabilidades, toma de decisiones, actividad investigadora y docente, sesiones clínicas conjuntas, creación/inicio, planificación, ventajas/desventajas del modelo y barreras/facilitadores en la implantación del modelo. Se describen sus características. Resultados. Analizamos 12 modelos de atención multidisciplinar en APs, implantados desde hace al menos 1-2 años, que globalmente pueden resumirse en 3 subtipos diferentes: presencial conjunto, presencial paralelo y circuito preferencial. La implantación de uno u otro modelo es consecuencia de la adaptación a las circunstancias del centro y profesionales. Una correcta planificación de la implantación es fundamental. La implicación y buena sintonía entre profesionales así como un acceso y criterios de derivación bien definidos son facilitadores muy importantes en la implantación de un modelo. La gestión de las agendas y la recogida de datos para medir resultados de salud de estos modelos son las principales barreras. Conclusiones. Existen distintos modelos de atención multidisciplinar implantados que tienen como objetivo intentar mejorar la atención del paciente con APs, la eficiencia del sistema y la colaboración entre especialistas (AU)

Objetive. To describe (structure, processes) of the multidisciplinary care models in psoriatic arthritis (PsA) in Spain, as well as barriers and facilitators of their implementation. Methods. A qualitative study was performed following structured interviews with 24 professionals (12 rheumatologists, 12 dermatologists who provide multidisciplinary care for patients with PsA). We collected data related to the hospital, department, population and multidisciplinary care model (type, physical and human resources, professional requirements, objectives, referral criteria, agendas, protocols, responsibilities, decision- making, research and education, clinical sessions, development and planning of the model, advantages and disadvantages of the model, barriers and facilitators in the implementation of the model. The models characteristics are described. Results. We analyzed 12 multidisciplinary care models in PsA, with at least 1-2 years of experience, and 3 subtypes of models, face-to-face, parallel, and preferential circuit. All are adapted to the hospital and professionals characteristics. A proper implementation planning is essential. The involvement and empathy between professionals and an access and well-defined referral criteria are important facilitators in the implementation of a model. The management of agendas and data collection to measure the multidisciplinary care models health outcomes are the main barriers. Conclusions. There are different multidisciplinary care models in PsA that can improve patient outcomes, system efficiency and collaboration between specialists (AU)

Dermatitis de contacto por Agave americana / Contact dermatitis from Agave americana

Numerosas especies de plantas y sus derivados pueden causar reacciones cutáneas por diversos mecanismos: dermatitis irritativa de contacto, dermatitis alérgica de contacto, urticaria de contacto y fotodermatitis. Se presenta un caso de dermatitis irritativa de contacto tras la exposición a la savia de Agave americana. Representa un cuadro cutáneo descrito en escasas ocasiones y que, aunque habitualmente se presenta como una erupción papulovesiculosa, en el paciente descrito aparece con lesiones purpúricas en la zona de contacto

Numerous plant species and their derivatives can cause skin reactions through a variety of mechanisms: irritative contact dermatitis, allergic contact dermatitis, contact urticaria and photodermatitis. We present a case of irritative contact dermatitis after exposure to the sap of Agave americana. The skin symptoms in this case have only been described on rare occasions; although this condition usually presents with a papulovesicular rash, in this patient it appeared as purpuric lesions in the contact area