Clinical course and disease burden of patients with generalized pustular psoriasis in Portugal: a multicenter retrospective cohort study.

OBJECTIVES: Generalized pustular psoriasis (GPP) is a rare, life-threatening skin inflammatory disorder. This study aimed to describe the disease course, treatment strategies, and healthcare utilization among patients with GPP in Portugal. METHODS: This multicentric, observational, retrospective study included consecutive adult patients with GPP undergoing a dermatology evaluation in different reporting institutions by experienced dermatologists between 2002 and 2023. RESULTS: A total of 59 patients were assessed. Most of the cohort had a previous history of plaque psoriasis (71%) and 83% presented at least one comorbidity. At the initial encounter, 64% of the cohort needed hospitalization. Systemic involvement was common, including fever (37%), and elevated white blood cell count and erythrocyte sedimentation rate/C-reactive protein (49%). Nearly, 73% of patients initiated systemic drugs, and 70% had to discontinue the first treatment. During the study, 98% of patients experienced at least one flare. At the last visit, 3.4% of patients had died, and 71.2% exhibited signs of active disease despite undergoing treatment. CONCLUSIONS: Our study demonstrates that GPP is a chronic, debilitating condition associated with systemic involvement, frequent flares, and hospitalizations, despite receiving multiple systemic treatments. Improved disease awareness and new treatments are needed to improve patient care and decrease the burden of the disease.

A influência do isolamento social devido à covid-19 na saúde mental do público infantil / The influence of social isolation due to COVID-19 on the mental health of children / La influencia del aislamiento social por el covid-19 en la salud mental de los niños

As infecções provocadas pelo coronavírus afetaram todos os países do mundo. Com a pandemia, surgiram muitos desafios a serem enfrentados para superar essa crise global. A quantidade de óbitos, o isolamento social e a incerteza das consequências futuras desse problema têm sido alguns dos principais fatores responsáveis por gerar instabilidade emocional nos indivíduos. Entre os grupos populacionais afetados, as crianças são as vítimas mais vulneráveis devido à prematuridade psicológica e à inabilidade de lidar com crises. Assim, foi realizada uma pesquisa de revisão integrativa nas plataformas Biblioteca Virtual em Saúde (BVS) e PubMed, a fim de investigar quais os efeitos da pandemia na saúde mental do público infantil. A amostra final foi composta de dez artigos. A partir da análise desses textos, pode-se perceber reflexos nas transformações de humor e de comportamento em crianças no contexto de isolamento social. O aumento da permanência em casa, a necessidade de se adaptar a atividades escolares virtuais, a privação do convívio social com outros da mesma idade e a maior exposição a telas nomearam os principais pontos percebidos. A pesquisa mostrou efeitos esperados da pandemia e identificou o despreparo das escolas e dos familiares em promover suporte emocional para esses menores. Dessa forma, é fundamental a realização de estudos sobre a temática com o objetivo de compreender as melhores medidas a serem adotadas para reduzir os efeitos negativos da pandemia na vida desse público.

Coronavirus infections affected all countries worldwide. With the pandemic came many challenges to be faced to overcome this global crisis. The number of deaths, the social isolation, and the uncertainty about the future consequences of this problem have been some of the main factors responsible for generating emotional instability in individuals. Among the affected population groups, children are the most vulnerable due to their psychological prematurity and inability to cope with crises. Thus, an integrative review research was carried out in the Virtual Health Library (BVS) and PubMed databases to investigate the effects of the pandemic on the mental health of children. The final sample comprised 10 studies. The text analysis shows reflexes in the transformations of mood and behavior in children in the context of social isolation. Increased home stay, the need to adapt to virtual school activities, the deprivation of social interaction with peers, and increased exposure to screens were the main points perceived. The research showed the expected effects of the pandemic and identified the unpreparedness of schools and family members to provide emotional support to these minors. Thus, it is essential to carry out further studies on the subject to understand the best measures to be adopted to reduce the negative effects of the pandemic in the lives of childrens.

Las infecciones provocadas por el coronavirus afectaron a todo el mundo. Junto a esta pandemia, han surgido muchos retos a enfrentarse para superar esta crisis global. El número de muertes, el aislamiento social y la incertidumbre de las consecuencias futuras de este problema han sido algunos de los principales factores responsables de generar inestabilidad emocional en los individuos. De los grupos afectados, los niños son las víctimas más vulnerables debido a la prematuridad psicológica y la incapacidad para hacer frente a las crisis. Así, se llevó a cabo una revisión integradora en las bases de datos Biblioteca Virtual en Salud (BVS) y PubMed, con el fin de investigar los efectos de la pandemia en la salud mental de los niños. La muestra final estuvo formada por diez artículos. A partir del análisis de estos textos, es posible notar transformaciones en el estado de ánimo y el comportamiento de los niños en el contexto del aislamiento. La permanencia en casa, la necesidad de adaptarse a las clases remotas, la privación de interacción social y el aumento del tiempo de uso de pantallas nombraron los principales puntos percibidos. La investigación mostró los efectos esperados de la pandemia y también identificó la falta de preparación de las escuelas y los miembros de la familia para promover el apoyo emocional de estos menores. Por tanto, es fundamental realizar estudios sobre el tema para comprender las mejores medidas que se debe tomar para reducir los efectos negativos de la pandemia en la vida de este público.

Thermal inclusions: how one spin can destroy a many-body localized phase.

Many-body localized (MBL) systems lie outside the framework of statistical mechanics, as they fail to equilibrate under their own quantum dynamics. Even basic features of MBL systems, such as their stability to thermal inclusions and the nature of the dynamical transition to thermalizing behaviour, remain poorly understood. We study a simple central spin model to address these questions: a two-level system interacting with strength J with N≫1 localized bits subject to random fields. On increasing J, the system transitions from an MBL to a delocalized phase on the vanishing scale Jc(N)∼1/N, up to logarithmic corrections. In the transition region, the single-site eigenstate entanglement entropies exhibit bimodal distributions, so that localized bits are either 'on' (strongly entangled) or 'off' (weakly entangled) in eigenstates. The clusters of 'on' bits vary significantly between eigenstates of the same sample, which provides evidence for a heterogeneous discontinuous transition out of the localized phase in single-site observables. We obtain these results by perturbative mapping to bond percolation on the hypercube at small J and by numerical exact diagonalization of the full many-body system. Our results support the arguments that the MBL phase is unstable in systems with short-range interactions and quenched randomness in dimensions d that are high but finite.This article is part of the themed issue 'Breakdown of ergodicity in quantum systems: from solids to synthetic matter'.

Cardiopulmonary Resuscitation in an Average Brazilian Intensive Care Unit: Should We Perform Less or Better?

INTRODUCTION:: Few data can be found about cardiac arrest in the intensive care unit outside reference centers in third world countries. OBJECTIVE:: To study epidemiology and prognostic factors associated with cardiac arrest in the intensive care unit (ICU) in an average Brazilian center. METHODS:: Between June 2011 and July 2014, 302 cases of cardiac arrest in the intensive care unit were prospectively evaluated in 273 patients (age: 68.9 ± 15 years) admitted in three mixed units. Data regarding cardiac arrest and cardiopulmonary resuscitation were collected in an "Utstein style" form and epidemiologic data was prospectively obtained. Factors associated with do not resuscitate orders, return of spontaneous circulation and survival were studied using binary logistic regression. Statistical package software used was SPSS 19.0 (IBM Inc., USA). RESULTS:: Among 302 cardiac arrests, 230 (76.3%) had their initial rhythm recorded and 141 (61.3%) was in asystole, 62 (27%) in pulseless electric activity (PEA) and 27 had a shockable rhythm (11.7%). In 109 (36.1%) cases, cardiac arrest had a suspected reversible cause. Most frequent suspected cardiac arrest causes were hypotension (n=98; 32.5%), multiple (19.2%) and hypoxemia (17.5%). Sixty (19.9%) cardiac arrests had do not resuscitate orders. Prior left ventricle dysfunction was the only predictor of do not resuscitate order (OR: 3.1 [CI=1.03-9.4]; P=0.04). Among patients that received cardiopulmonary resuscitation, 59 (24.4%) achieved return of spontaneous circulation and 12 survived to discharge (5.6%). Initial shockable rhythm was the only return of spontaneous circulation predictor (OR: 24.9 (2.4-257); P=0.007) and survival (OR: 4.6 (1.4-15); P=0.01). CONCLUSION:: Cardiopulmonary resuscitation rate was high considering ICU patients, so was mortality. Prior left ventricular dysfunction was a predictor of do not resuscitate order. Initial shockable rhythm was a predictor of return of spontaneous circulation and survival.

Cardiopulmonary resuscitation in an average brazilian intensive care unit: should we perform less or better?

Abstract Introduction: Few data can be found about cardiac arrest in the intensive care unit outside reference centers in third world countries. Objective: To study epidemiology and prognostic factors associated with cardiac arrest in the intensive care unit (ICU) in an average Brazilian center. Methods: Between June 2011 and July 2014, 302 cases of cardiac arrest in the intensive care unit were prospectively evaluated in 273 patients (age: 68.9 ± 15 years) admitted in three mixed units. Data regarding cardiac arrest and cardiopulmonary resuscitation were collected in an "Utstein style" form and epidemiologic data was prospectively obtained. Factors associated with do not resuscitate orders, return of spontaneous circulation and survival were studied using binary logistic regression. Statistical package software used was SPSS 19.0 (IBM Inc., USA). Results: Among 302 cardiac arrests, 230 (76.3%) had their initial rhythm recorded and 141 (61.3%) was in asystole, 62 (27%) in pulseless electric activity (PEA) and 27 had a shockable rhythm (11.7%). In 109 (36.1%) cases, cardiac arrest had a suspected reversible cause. Most frequent suspected cardiac arrest causes were hypotension (n=98; 32.5%), multiple (19.2%) and hypoxemia (17.5%). Sixty (19.9%) cardiac arrests had do not resuscitate orders. Prior left ventricle dysfunction was the only predictor of do not resuscitate order (OR: 3.1 [CI=1.03-9.4]; P=0.04). Among patients that received cardiopulmonary resuscitation, 59 (24.4%) achieved return of spontaneous circulation and 12 survived to discharge (5.6%). Initial shockable rhythm was the only return of spontaneous circulation predictor (OR: 24.9 (2.4-257); P=0.007) and survival (OR: 4.6 (1.4-15); P=0.01). Conclusion: Cardiopulmonary resuscitation rate was high considering ICU patients, so was mortality. Prior left ventricular dysfunction was a predictor of do not resuscitate order. Initial shockable rhythm was a predictor of return of spontaneous circulation and survival.

Many-body localization in periodically driven systems.

We consider disordered many-body systems with periodic time-dependent Hamiltonians in one spatial dimension. By studying the properties of the Floquet eigenstates, we identify two distinct phases: (i) a many-body localized (MBL) phase, in which almost all eigenstates have area-law entanglement entropy, and the eigenstate thermalization hypothesis (ETH) is violated, and (ii) a delocalized phase, in which eigenstates have volume-law entanglement and obey the ETH. The MBL phase exhibits logarithmic in time growth of entanglement entropy when the system is initially prepared in a product state, which distinguishes it from the delocalized phase. We propose an effective model of the MBL phase in terms of an extensive number of emergent local integrals of motion, which naturally explains the spectral and dynamical properties of this phase. Numerical data, obtained by exact diagonalization and time-evolving block decimation methods, suggest a direct transition between the two phases.

Birt-Hogg-Dubé syndrome.

A 45-year-old woman with a history of renal carcinoma was observed for facial, cervical and truncal flesh-colored papules. Relatives had similar skin findings and a brother had repeated episodes of pneumothorax. The computerized tomography scan revealed multiple cysts on both lungs. A skin biopsy revealed a perifollicular fibroma. The clinical diagnosis of Birt-Hogg-Dubé syndrome (BHDS) was corroborated by identification of a novel frameshift c.573delGAinsT (p.G191fsX31) mutation in heterozygosity on exon 6 of the folliculin gene. The presence of multiple and typical benign hair follicle tumors highlights the role of the dermatologist in the diagnosis of this rare genodermatosis that is associated with an increased risk of renal cell cancer and pulmonary cysts, warranting personal and familial follow-up and counseling.

Birt-Hogg-Dubé syndrome / Sindrome de Birt-Hogg-Dube

A 45-year-old woman with a history of renal carcinoma was observed for facial, cervical and truncal flesh-colored papules. Relatives had similar skin findings and a brother had repeated episodes of pneumothorax. The computerized tomography scan revealed multiple cysts on both lungs. A skin biopsy revealed a perifollicular fibroma. The clinical diagnosis of Birt-Hogg-Dubé syndrome (BHDS) was corroborated by identification of a novel frameshift c.573delGAinsT (p.G191fsX31) mutation in heterozygosity on exon 6 of the folliculin gene. The presence of multiple and typical benign hair follicle tumors highlights the role of the dermatologist in the diagnosis of this rare genodermatosis that is associated with an increased risk of renal cell cancer and pulmonary cysts, warranting personal and familial follow-up and counseling.

Uma mulher de 45 anos com história de carcinoma renal foi observada por pápulas cor da pele, faciais, cervicais e tronculares. Referia história familiar de achados cutâneos semelhantes e irmão com episódios repetidos de pneumotórax. Identificaram-se múltiplos quistos pulmonares por tomografia computorizada. Uma biópsia cutânea revelou fibroma perifolicular. O diagnóstico clínico de síndrome de Birt-Hogg-Dubé (BHDS) foi contudo corroborado pela identificação de uma nova mutação frameshift c.573delGAinsT (p.G191fsX31) em heterozigotia no exão 6 do gene da foliculina. A presença de múltiplos e típicos tumores benignos do folículo piloso, realça o papel do dermatologista no diagnóstico desta rara genodermatose, que está associada a um risco aumentado de tumores de células renais e cistos pulmonares, exigindo seguimento e aconselhamento pessoal e familiar.

Intraepidermal epidermotropic metastatic melanoma: a clinical and histopathological mimicker of melanoma in situ occurring in multiplicity.

The distinction between primary melanoma and melanoma metastatic to the skin has major prognostic implications. We report a case of a 67-year-old male with a diagnosis of a superficial spreading melanoma (stage IB) rendered 6 years earlier who presented clinically with an atypical nevus on his left thigh. Histopathological examination showed an intraepidermal melanocytic proliferation that was interpreted as melanoma in situ. Subsequently, 45 additional pigmented macules appeared in crops over a 9-month period. Clinically and dermoscopically, these lesions were extremely polymorphic. Histopathological findings were compatible with melanoma in situ, as each lesion consisted of a wholly intraepidermal proliferation of markedly atypical melanocytes arranged singly and in nests. A complete gastrointestinal study showed multiple pigmented metastatic lesions throughout the stomach and small bowel, which supported a diagnosis of metastatic melanoma with gastrointestinal and epidermotropic skin involvement. Monosomy of chromosome 9 and a BRAF V600E mutation were detected in the primary tumor sample and in macro-dissected secondary lesions. No CDKN2A or CDK4 germline mutations were found. Intraepidermal epidermotropic metastases of melanoma have been rarely described in literature. In this case, histopathology alone was insufficient to distinguish metastatic melanoma from multiple in situ melanomas. The recognition of epidermotropic metastases should be based on the correlation between clinical, dermoscopic, histopathological and molecular findings.

Borderline CD30+ cutaneous lymphoproliferative disorder: report of a case with expression of cytotoxic markers and response to clarithromycin.

CD30+ cutaneous lymphoproliferative disorders (CLPDs) are usually characterized by a benign clinical course. The prognostic value of cytotoxic markers in these lymphomas has not been evaluated in large series. We describe a case of borderline CD30+ CLPD with cytotoxic phenotype, presenting in a 22-year-old male patient as an ulcer on the forearm. He reported having had similar ulcers on the buttock and thigh that spontaneously regressed over the course of 1 year. The lesion resolved with a single course of clarithromycin; a subsequent lesion, too, responded to clarithromycin, and no recurrences or systemic involvement have been documented in the 9-month follow-up. A conservative approach in the management of CD30+ CLPD is recommended. We believe that the anti-inflammatory and apoptotic effects of clarithromycin on T cells may have hastened the remission process.

Merkel cell carcinoma: an unusual indication for isolated limb perfusion.

Isolated limb perfusion (ILP) is a surgical technique that enables the administration of high-dose chemotherapy while minimizing serious systemic side effects. The clinical value and indications are well established for skin and soft tissue tumors on limbs. For skin tumors, this technique is mainly indicated for melanoma with in-transit metastasis. For soft tissue tumors--sarcoma and osteosarcoma--it is useful as a palliative technique to reduce the tumoral mass. Limb perfusion can also be an option in other tumors, such as advanced stage squamous cell carcinoma or Merkel cell carcinoma. We present a case report of a 68-year-old man with Merkel cell carcinoma on the right tibiotarsical region, with in-transit metastasis throughout the whole lower limb. Regional chemotherapy involving ILP with melphalan and tumor necrosis factor-alpha (TNFalpha) was performed in order to avoid amputation; the primary tumor was not excised. A steady regression of the disease was observed, with complete resolution of all visible in-transit metastases at the 45th day post-perfusion. However, systemic metastasis leading to fatal outcome occurred 4 months later. Although there was no change in the patient's prognosis, ILP was able to avoid limb amputation as it controlled local-regional disease and produced complete regional remission. The addition of TNFalpha to melphalan in ILP appears to produce greater efficacy in the treatment of patients with bulky tumors or a large number of in-transit metastases.