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1.
PLoS Biol ; 21(4): e3001820, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-37071598

RESUMO

Movement is critical to animal survival and, thus, biodiversity in fragmented landscapes. Increasing fragmentation in the Anthropocene necessitates predictions about the movement capacities of the multitude of species that inhabit natural ecosystems. This requires mechanistic, trait-based animal locomotion models, which are sufficiently general as well as biologically realistic. While larger animals should generally be able to travel greater distances, reported trends in their maximum speeds across a range of body sizes suggest limited movement capacities among the largest species. Here, we show that this also applies to travel speeds and that this arises because of their limited heat-dissipation capacities. We derive a model considering how fundamental biophysical constraints of animal body mass associated with energy utilisation (i.e., larger animals have a lower metabolic energy cost of locomotion) and heat-dissipation (i.e., larger animals require more time to dissipate metabolic heat) limit aerobic travel speeds. Using an extensive empirical dataset of animal travel speeds (532 species), we show that this allometric heat-dissipation model best captures the hump-shaped trends in travel speed with body mass for flying, running, and swimming animals. This implies that the inability to dissipate metabolic heat leads to the saturation and eventual decrease in travel speed with increasing body mass as larger animals must reduce their realised travel speeds in order to avoid hyperthermia during extended locomotion bouts. As a result, the highest travel speeds are achieved by animals of intermediate body mass, suggesting that the largest species are more limited in their movement capacities than previously anticipated. Consequently, we provide a mechanistic understanding of animal travel speed that can be generalised across species, even when the details of an individual species' biology are unknown, to facilitate more realistic predictions of biodiversity dynamics in fragmented landscapes.


Assuntos
Temperatura Alta , Corrida , Animais , Ecossistema , Locomoção , Tamanho Corporal
3.
Ecol Lett ; 27(9): e14522, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39354907

RESUMO

Information transmission among species is a fundamental aspect of natural ecosystems that faces significant disruption from rapidly growing anthropogenic sensory pollution. Understanding the constraints of information flow on species' trophic interactions is often overlooked due to a limited comprehension of the mechanisms of information transmission and the absence of adequate analytical tools. To fill this gap, we developed a sensory information-constrained functional response (IFR) framework, which accounts for the information transmission between predator and prey. Through empirical evaluation, the IFR provided a biologically grounded explanation for the systematic variation of functional responses. Specifically, it posits that the variation of different functional-response shapes, associated with community stability, is attributable to limitations in sensory information transmission among species. This not only deepens our mechanistic understanding of species interactions but also elucidates how anthropogenic activities are reshaping species interactions and community dynamics by disrupting information exchange through sensory pollution.


Assuntos
Cadeia Alimentar , Animais , Ecossistema , Modelos Biológicos , Comportamento Predatório
4.
Hematol Oncol ; 42(1): e3215, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37649350

RESUMO

Primary cutaneous B-cell lymphomas (PCBCLs) are lymphoproliferative disorders that appear on the skin without evidence of extracutaneous manifestations at the time of diagnosis. There is a lack of evidence-based guidelines for their clinical management due to the availability of very few large scale studies and controlled clinical trials. Here we present and discuss a series of major unmet clinical needs (UCNs) in the management of PCBCLs by a panel of 16 experts involved in research and clinical practice of PCBCL. The Panel produced recommendations on the appropriateness of the clinical decisions concerning the identified clinical needs and proposed research for improving the knowledge needed to solve them. Recommendations and proposals were achieved by multiple-step formalized procedures to reach a consensus after a comprehensive analysis of the scientific literature. Recommendations and proposals lay in the domain of classification uncertainties of PCBCL, optimization of diagnosis, optimization of prognosis, optimization of staging and critical issues on therapeutic strategies with particular focus on new treatments. These recommendations are intended for use not only by experts but above all by dermatologists and hematologists with limited experience in the field of PCBCLs as well as general practitioners.


Assuntos
Linfoma de Células B , Neoplasias Cutâneas , Humanos , Linfoma de Células B/diagnóstico , Linfoma de Células B/terapia , Linfoma de Células B/patologia , Consenso , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/terapia , Neoplasias Cutâneas/patologia , Prognóstico
5.
Br J Dermatol ; 191(2): 233-242, 2024 Jul 16.
Artigo em Inglês | MEDLINE | ID: mdl-38595050

RESUMO

BACKGROUND: Lymphomatoid papulosis (LyP) is a rare cutaneous T-cell lymphoproliferative disorder. Comprehensive data on LyP in the paediatric population are scarce. OBJECTIVES: To characterize the epidemiological, clinical, histopathological and prognostic features of paediatric LyP. METHODS: This was a retrospective multicentre international cohort study that included 87 children and adolescents with LyP diagnosed between 1998 and 2022. Patients aged ≤ 18 years at disease onset were included. LyP diagnosis was made in each centre, based on clinicopathological correlation. RESULTS: Eighty-seven patients from 12 centres were included. Mean age at disease onset was 7.0 years (range 3 months-18 years) with a male to female ratio of 2 : 1. Mean time between the onset of the first cutaneous lesions and diagnosis was 1.3 years (range 0-14). Initial misdiagnosis concerned 26% of patients. LyP was most often misdiagnosed as pityriasis lichenoides et varioliformis acuta, insect bites or mollusca contagiosa. Erythematous papules or papulonodules were the most frequent clinical presentation. Pruritus was specifically mentioned in 21% of patients. The main histological subtype was type A in 55% of cases. When analysed, monoclonal T-cell receptor rearrangement was found in 77% of skin biopsies. The overall survival rate was 100%, with follow-up at 5 years available for 33 patients and at 15 years for 8 patients. Associated haematological malignancy (HM) occurred in 10% of cases (n = 7/73), including four patients with mycosis fungoides, one with primary cutaneous anaplastic large cell lymphoma (ALCL), one with systemic ALCL and one with acute myeloid leukaemia. If we compared incidence rates of cancer with the world population aged 0-19 years from 2001 to 2010, we estimated a significantly higher risk of associated malignancy in general, occurring before the age of 19 years (incidence rate ratio 87.49, 95% confidence interval 86.01-88.99). CONCLUSIONS: We report epidemiological data from a large international cohort of children and adolescents with LyP. Overall, the disease prognosis is good, with excellent survival rates for all patients. Owing to an increased risk of associated HM, long-term follow-up should be recommended for patients with LyP.


Lymphomatoid papulosis is a very rare skin condition caused by an abnormal increase in white blood cells (called 'lymphocytes') in the skin. The condition rarely affects children, so most of the scientific data published about this disease focuses on adults. This study involved 12 academic dermatology centres in Europe, the Middle East and North America, and gathered data from about 87 children who presented with symptoms of lymphomatoid papulosis before the age of 19 years. The aim of this study was to better describe this disease in the paediatric population and discuss its treatment options and evolution. We found that the presentation of the disease in children is roughly the same as in adults. Safe and effective treatment options exist. The disease is not life threatening, but it requires investigation by a dermatologist, both to make a careful diagnosis and to monitor it as sometimes associated cancers that originate from blood cells can occur, mostly on the skin.


Assuntos
Papulose Linfomatoide , Neoplasias Cutâneas , Humanos , Papulose Linfomatoide/patologia , Papulose Linfomatoide/epidemiologia , Masculino , Estudos Retrospectivos , Criança , Feminino , Adolescente , Pré-Escolar , Lactente , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/mortalidade , Idade de Início , Prognóstico , Erros de Diagnóstico/estatística & dados numéricos , Pitiríase Liquenoide/epidemiologia , Pitiríase Liquenoide/patologia , Pitiríase Liquenoide/diagnóstico , Mordeduras e Picadas de Insetos/epidemiologia , Mordeduras e Picadas de Insetos/complicações , Molusco Contagioso/epidemiologia , Molusco Contagioso/patologia , Molusco Contagioso/diagnóstico
6.
Artigo em Inglês | MEDLINE | ID: mdl-38650545

RESUMO

BACKGROUND: Primary cutaneous lymphomas are neoplasms of the immune system with a distinct tropism for the skin and an absence of extracutaneous manifestations at the time of diagnosis. Studies focusing on cutaneous lymphomas in children and adolescents remain scarce and often do not encompass the rare subtypes. OBJECTIVES: To address this knowledge gap by describing the clinical, histological and molecular characteristics of a large group of paediatric patients affected by primary cutaneous lymphoma. We also provided the Paediatric Primary Cutaneous Lymphoma Atlas that illustrates the clinicopathological spectrum of observed presentations, in the hope of supporting other physicians in the diagnostic process. METHODS: Retrospective chart review of paediatric patients diagnosed with primary cutaneous lymphomas between 1980 and 2022 at the Paediatric Dermatology Unit of Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan. RESULTS: A total of 101 patients (58 males, 43 females) met the inclusion criteria. The most common subtypes were lymphomatoid papulosis (n = 48) and mycosis fungoides (n = 31). These were followed by primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorders (n = 7), primary cutaneous anaplastic large-cell lymphomas (n = 5), primary cutaneous marginal zone B-cell lymphomas (n = 3), primary cutaneous follicle centre lymphomas (n = 2), subcutaneous panniculitis-like T-cell lymphomas (n = 2), primary cutaneous peripheral T-cell lymphoma not otherwise specified (n = 1), primary cutaneous precursor B-lymphoblastic lymphoma (n = 1) and Sézary syndrome (n = 1). Clinical follow-up data covering a median of 70.8 months (range 1-324) were available for 74 patients, of whom three died due to cutaneous lymphoma. CONCLUSIONS: Our findings shed light on the peculiar aspects and long-term outcomes of paediatric cutaneous lymphomas, particularly emphasizing their distinctive features in comparison to their adult counterparts and exploring the less common subtypes. Further larger-scale studies are warranted to better characterize these entities and to achieve a more rapid and accurate diagnosis.

7.
J Cutan Pathol ; 50(5): 420-424, 2023 May.
Artigo em Inglês | MEDLINE | ID: mdl-36764679

RESUMO

The association between acanthosis nigricans (AN) and mycosis fungoides (MF) has rarely been described, but it is known that MF may appear as AN-like vegetating and papillomatous plaques in skin folds, or may be associated with paraneoplastic AN. There have also been recent descriptions of a form of "intertriginous MF" that is characterized by skin fold involvement and the expression of T follicular helper (TFH) markers, and that often has an aggressive course. We describe the case of a 48-year-old man affected by MF associated with AN, whose lesions were characterized by a TFH immunophenotype and the expression of the GATA-3 nuclear master regulator that may be related to a TFH-2 subpopulation or possible disease progression.


Assuntos
Acantose Nigricans , Micose Fungoide , Neoplasias Cutâneas , Humanos , Acantose Nigricans/complicações , Acantose Nigricans/patologia , Neoplasias Cutâneas/patologia , Micose Fungoide/patologia , Fenótipo , Linfócitos T Auxiliares-Indutores/patologia
8.
Dermatology ; 239(4): 584-591, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37075721

RESUMO

BACKGROUND: COronaVIrus Disease 19 (COVID-19) is associated with a wide spectrum of skin manifestations, but SARS-CoV-2 RNA in lesional skin has been demonstrated only in few cases. OBJECTIVE: The objective of this study was to demonstrate SARS-CoV-2 presence in skin samples from patients with different COVID-19-related cutaneous phenotypes. METHODS: Demographic and clinical data from 52 patients with COVID-19-associated cutaneous manifestations were collected. Immunohistochemistry and digital PCR (dPCR) were performed in all skin samples. RNA in situ hybridization (ISH) was used to confirm the presence of SARS-CoV-2 RNA. RESULTS: Twenty out of 52 (38%) patients presented SARS-CoV-2 positivity in the skin. Among these, 10/52 (19%) patients tested positive for spike protein on immunohistochemistry, five of whom had also positive testing on dPCR. Of the latter, one tested positive both for ISH and ACE-2 on immunohistochemistry while another one tested positive for nucleocapsid protein. Twelve patients showed positivity only for nucleocapsid protein on immunohistochemistry. CONCLUSIONS: SARS-CoV-2 was detected only in 38% of patients, without any association with a specific cutaneous phenotype, suggesting that the pathophysiology of cutaneous lesions mostly depends on the activation of the immune system. The combination of spike and nucleocapsid immunohistochemistry has higher diagnostic yield than dPCR. Skin persistence of SARS-CoV-2 may depend on timing of skin lesions, viral load, and immune response.


Assuntos
COVID-19 , Humanos , COVID-19/diagnóstico , SARS-CoV-2/genética , SARS-CoV-2/metabolismo , Imuno-Histoquímica , RNA Viral/análise , RNA Viral/metabolismo , Proteínas do Nucleocapsídeo/genética , Proteínas do Nucleocapsídeo/metabolismo , Reação em Cadeia da Polimerase , Biópsia , Teste para COVID-19
9.
Artigo em Inglês | MEDLINE | ID: mdl-37016977

RESUMO

Indeterminate cell histiocytosis (ICH) is a very rare histiocytic disorder, primarily involving the skin. It affects more frequently adults, often presenting with a generalized papular eruption, and needs to be differentiated from other neoplastic, paraneoplastic, and infectious diseases through clinical and histological examination. The knowledge on ICH is limited to case reports and small series. Thus, the lack of larger multicentric studies has prevented recognizing and addressing the specific clinical need of the entity. In this systematic review, we comprehensively analysed the medical literature describing histologically-confirmed cases of ICH and divided the patients into epidemiologically and clinically different groups. We demonstrate that ICH in adulthood is strongly associated with the development of haematological (and especially myeloid) neoplasms. In this subset of patients, we identify blastic morphology of neoplastic cells as a novel independent prognostic factor and an early histopathological predictor of an associated myeloid neoplasm. Moreover, we highlight that even though ICH may also present in childhood, these patients often show indolent behaviour. Genetically, ICH emerges as a heterogeneous condition. While patients with associated myeloid neoplasms are enriched in pERK pathway gene mutations, in others a specific ETV3::NCOA2 rearrangement is described. We finally reviewe the nosology of ICH since its first description, its possible cell of origin, and summarize the therapeutic options reported for each different clinical subgroup. With this work, we hope to foster studies on rare cutaneous histiocytosis and their comprehensive multidisciplinary characterization.

10.
Ecol Lett ; 25(5): 1225-1236, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-35286010

RESUMO

Despite intensive research on species dissimilarity patterns across communities (i.e. ß-diversity), we still know little about their implications for variation in food-web structures. Our analyses of 50 lake and 48 forest soil communities show that, while species dissimilarity depends on environmental and spatial gradients, these effects are only weakly propagated to the networks. Moreover, our results show that species and food-web dissimilarities are consistently correlated, but that much of the variation in food-web structure across spatial, environmental, and species gradients remains unexplained. Novel food-web assembly models demonstrate the importance of biotic filtering during community assembly by (1) the availability of resources and (2) limiting similarity in species' interactions to avoid strong niche overlap and thus competitive exclusion. This reveals a strong signature of biotic filtering processes during local community assembly, which constrains the variability in structural food-web patterns across local communities despite substantial turnover in species composition.


Assuntos
Biodiversidade , Florestas , Ecossistema , Cadeia Alimentar , Solo
11.
Eur J Immunol ; 51(7): 1660-1671, 2021 07.
Artigo em Inglês | MEDLINE | ID: mdl-33811642

RESUMO

New treatments are needed for patients with cutaneous T-cell lymphoma (CTCL), particularly for advanced mycosis fungoides (MF) and Sezary syndrome (SS). The immunopathology of MF and SS is complex, but recent advances in tumor microenvironment understanding have identified CCR4 as a promising therapeutic target. CCR4 is widely expressed on malignant T cells and Tregs in the skin and peripheral blood of patients with MF and SS. The interaction of CCR4 with its dominant ligands CCL17 and CCL22 plays a critical role in the development and progression of CTCL, facilitating the movement into, and accumulation of, CCR4-expressing T cells in the skin, and recruiting CCR4-expressing Tregs into the tumor microenvironment. Expression of CCR4 is upregulated at all stages of MF and in SS, increasing with advancing disease. Several CCR4-targeted therapies are being evaluated, including "chemotoxins" targeting CCR4 via CCL17, CCR4-directed chimeric antigen receptor-modified T-cell therapies, small-molecule CCR4 antagonists, and anti-CCR4 monoclonal antibodies. Only one is currently approved: mogamulizumab, a defucosylated, fully humanized, anti-CCR4, monoclonal antibody for the treatment of relapsed/refractory MF and SS. Clinical trial da1ta confirm that mogamulizumab is an effective and well-tolerated treatment for relapsed/refractory MF or SS, demonstrating the clinical value of targeting CCR4.


Assuntos
Antineoplásicos Imunológicos/farmacologia , Linfoma Cutâneo de Células T/tratamento farmacológico , Linfoma Cutâneo de Células T/metabolismo , Receptores CCR4/metabolismo , Animais , Humanos , Pele/efeitos dos fármacos , Pele/metabolismo , Microambiente Tumoral/efeitos dos fármacos
12.
Haematologica ; 107(3): 702-714, 2022 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-33792220

RESUMO

Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma (pcAECyTCL) is a rare variant of cutaneous T-cell lymphoma with an aggressive clinical course and a very poor prognosis. Until now, neither a systematic characterization of genetic alterations driving pcAECyTCL has been performed, nor effective therapeutic regimes for patients have been defined. Here, we present the first highresolution genetic characterization of pcAECyTCL by using wholegenome and RNA sequencing. Our study provides a comprehensive description of genetic alterations (i.e., genomic rearrangements, copy number alterations and small-scale mutations) with pathogenic relevance in this lymphoma, including events that recurrently impact genes with important roles in the cell cycle, chromatin regulation and the JAKSTAT pathway. In particular, we show that mutually exclusive structural alterations involving JAK2 and SH2B3 predominantly underlie pcAECyTCL. In line with the genomic data, transcriptome analysis uncovered upregulation of the cell cycle, JAK2 signaling, NF-κB signaling and a high inflammatory response in this cancer. Functional studies confirmed oncogenicity of JAK2 fusions identified in pcAECyTCL and their sensitivity to JAK inhibitor treatment. Our findings strongly suggest that overactive JAK2 signaling is a central driver of pcAECyTCL, and consequently, patients with this neoplasm would likely benefit from therapy with JAK2 inhibitors such as Food and Drug Adminstration-approved ruxolitinib.


Assuntos
Linfoma Cutâneo de Células T , Neoplasias Cutâneas , Linfócitos T CD8-Positivos/metabolismo , Humanos , Janus Quinase 2/genética , Linfoma Cutâneo de Células T/genética , Pele/patologia , Neoplasias Cutâneas/tratamento farmacológico , Neoplasias Cutâneas/genética , Neoplasias Cutâneas/patologia , Linfócitos T Citotóxicos/metabolismo
13.
Br J Dermatol ; 186(5): 887-897, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-34988968

RESUMO

BACKGROUND: The differential diagnosis of atypical dermal nonepidermotropic CD8+ lymphocytic infiltrates includes a heterogeneous spectrum of lymphoproliferations with overlapping histological and phenotypic features, but divergent clinical manifestations and prognoses. As these neoplasms are rare, more data on their clinicopathological presentation and course are needed. OBJECTIVES: To assess the clinical, histological and immunophenotypic features; outcomes of; and differences between dermal CD8+ lymphoproliferations. METHODS: Retrospective analysis of a series of 46 patients and biopsies by the international EORTC Cutaneous Lymphoma Group. RESULTS: The dermal CD8+ lymphoproliferations (n = 46) could be assigned to one of three groups: (i) cutaneous acral CD8+ T-cell lymphoma (n = 31), characterized mostly by a solitary nodule arising at acral sites, a monotonous dermal infiltrate of small-to-medium-sized CD8+ lymphocytes with a characteristic dot-like pattern of CD68, a low proliferation rate and an excellent prognosis; (ii) primary cutaneous CD8+ peripheral T-cell lymphoma, unspecified/NOS (n = 11), presenting with one or multiple rapidly evolving tumours, mostly medium-sized pleomorphic CD8+ tumour cells with expression of several cytotoxic markers, and high proliferative activity; and (iii) cutaneous CD8+ lymphoproliferations (n = 4), associated with congenital immunodeficiency syndromes in two patients with persisting localized or disseminated violaceous to brownish plaques on the extremities, a histiocyte-rich infiltrate of mostly small CD8+ lymphocytes with subtle atypia and a protracted course; and papular CD8+ eruptions in two patients with acquired immunosuppression. CONCLUSIONS: A constellation of distinct clinical, histopathological and phenotypic features allows discrimination and assignment of dermal CD8+ infiltrates into distinct disease entities. Primary cutaneous acral CD8+ lymphoma, assigned a provisional category in current lymphoma classifications, is a distinct and reproducible entity. A correct diagnosis is essential to avoid unnecessarily aggressive treatment for indolent CD8+ lymphoproliferations and to identify cases with underlying immuno-deficiency or potential for dismal outcome.


Assuntos
Linfoma Cutâneo de Células T , Neoplasias Cutâneas , Linfócitos T CD8-Positivos/patologia , Humanos , Linfoma Cutâneo de Células T/diagnóstico , Linfoma Cutâneo de Células T/patologia , Prognóstico , Estudos Retrospectivos , Neoplasias Cutâneas/patologia
14.
Cancer ; 127(7): 1091-1101, 2021 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-33270908

RESUMO

BACKGROUND: Patients with cancer are considered at high risk for the novel respiratory illness coronavirus disease 2019 (COVID-19). General measures to keep COVID-19-free cancer divisions have been adopted worldwide. The objective of this study was to evaluate the efficacy of triage to identify COVID-19 among patients with cancer. METHODS: From March 20 to April 17, 2020, data were collected from patients who were treated or followed at the authors' institution in a prospective clinical trial. The primary endpoint was to estimate the cumulative incidence of COVID-19-positive patients who were identified using a triage process through the aid of medical and patient questionnaires. Based on a diagnostic algorithm, patients with suspect symptoms underwent an infectious disease specialist's evaluation and a COVID-19 swab. Serologic tests were proposed for patients who had symptoms or altered laboratory tests that did not fall into the diagnostic algorithm but were suspicious for COVID-19. RESULTS: Overall, 562 patients were enrolled. Six patients (1%) were diagnosed with COVID-19, of whom 4 (67%) had the disease detected through telehealth triage, and 2 patients (33%) without suspect symptoms at triage had the disease detected later. Seventy-one patients (13%) had suspect symptoms and/or altered laboratory tests that were not included in the diagnostic algorithm and, of these, 47 patients (73%) underwent testing for severe acute respiratory syndrome coronavirus 2 antibody: 6 (13%) were positive for IgG (n = 5) or for both IgM and IgG (n = 1), and antibody tests were negative in the remaining 41 patients. CONCLUSIONS: The triage process had a positive effect on the detection of COVID-19 in patients with cancer. Telehealth triage was helpful in detecting suspect patients and to keep a COVID-19-free cancer center. The overall incidence of COVID-19 diagnosis (1%) and antibody positivity (13%) in patients with suspect symptoms was similar to that observed in the general population.


Assuntos
Teste para COVID-19/estatística & dados numéricos , COVID-19/diagnóstico , Neoplasias/terapia , Triagem/estatística & dados numéricos , Adulto , Idoso , Idoso de 80 Anos ou mais , COVID-19/complicações , COVID-19/virologia , Teste para COVID-19/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias/complicações , Neoplasias/diagnóstico , Estudos Prospectivos , Reprodutibilidade dos Testes , SARS-CoV-2/fisiologia , Sensibilidade e Especificidade , Triagem/métodos
15.
Blood ; 133(16): 1703-1714, 2019 04 18.
Artigo em Inglês | MEDLINE | ID: mdl-30635287

RESUMO

Primary cutaneous lymphomas are a heterogeneous group of T- and B-cell lymphomas that present in the skin with no evidence of extracutaneous disease at the time of diagnosis. The 2005 World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) consensus classification has served as a golden standard for the diagnosis and classification of these conditions. In September 2018, an updated version of the WHO-EORTC was published in the fourth edition of the WHO Classification of Skin Tumours Blue Book. In this classification, primary cutaneous acral CD8+ T-cell lymphoma and Epstein-Barr virus positive (EBV+) mucocutaneous ulcer are included as new provisional entities, and a new section on cutaneous forms of chronic active EBV disease has been added. The term "primary cutaneous CD4+ small/medium T-cell lymphoma" was modified to "primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder" because of its indolent clinical behavior and uncertain malignant potential. Modifications have also been made in the sections on lymphomatoid papulosis, increasing the spectrum of histologic and genetic types, and primary cutaneous marginal zone lymphomas recognizing 2 different subtypes. Herein, the characteristic features of these new and modified entities as well as the results of recent molecular studies with diagnostic, prognostic, and/or therapeutic significance for the different types of primary cutaneous lymphomas are reviewed. An update of the frequency and survival of the different types of primary cutaneous lymphomas is provided.


Assuntos
Linfoma Cutâneo de Células T/classificação , Neoplasias Cutâneas/classificação , Herpesvirus Humano 4 , Humanos , Linfoma de Células B , Linfoma Cutâneo de Células T/diagnóstico , Linfoma Cutâneo de Células T/terapia , Técnicas de Diagnóstico Molecular/tendências , Prognóstico , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/terapia , Terapêutica/tendências , Organização Mundial da Saúde
16.
J Am Acad Dermatol ; 84(5): 1356-1363, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33476725

RESUMO

BACKGROUND: COVID-19 is associated with a wide range of skin manifestations. OBJECTIVE: To describe the clinical characteristics of COVID-19-associated skin manifestations and explore the relationships among the 6 main cutaneous phenotypes and systemic findings. METHODS: Twenty-one Italian Dermatology Units were asked to collect the demographic, clinical, and histopathologic data of 200 patients with COVID-19-associated skin manifestations. The severity of COVID-19 was classified as asymptomatic, mild, moderate, or severe. RESULTS: A chilblain-like acral pattern was significantly associated with a younger age (P < .0001) and, after adjusting for age, significantly associated with less severe COVID-19 (P = .0009). However, the median duration of chilblain-like lesions was significantly longer than that of the other cutaneous manifestations taken together (P < .0001). Patients with moderate/severe COVID-19 were more represented than those with asymptomatic/mild COVID-19 among the patients with cutaneous manifestations other than chilblain-like lesions, but only the confluent erythematous/maculo-papular/morbilliform phenotype was significantly associated with more severe COVID-19 (P = .015), and this significance disappeared after adjustment for age. LIMITATIONS: Laboratory confirmation of COVID-19 was not possible in all cases. CONCLUSIONS: After adjustment for age, there was no clear-cut spectrum of COVID-19 severity in patients with COVID-19-related skin manifestations, although chilblain-like acral lesions were more frequent in younger patients with asymptomatic/pauci-symptomatic COVID-19.


Assuntos
COVID-19/diagnóstico , Dermatopatias Virais/diagnóstico , Adulto , Idade de Início , Idoso , Pérnio/virologia , Humanos , Itália , Masculino , Pessoa de Meia-Idade , SARS-CoV-2 , Índice de Gravidade de Doença , Dermatopatias Virais/patologia
17.
J Cutan Pathol ; 48(5): 637-643, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33188581

RESUMO

Mixed histiocytoses are a rare and recently recognized subset of histiocytic disorders that may involve the skin, characterized by the synchronous or metachronous development of lesions with Langerhans and/or non-Langerhans cell histiocytosis histopathological features. Around 10% of patients diagnosed with histiocytosis may develop a hematological malignancy, often with dramatic prognostic consequences. We hereby describe the exceptional case of a patient developing a MAP2K1-driven mixed histiocytosis with Langerhans cell histiocytosis, Rosai-Dorfman-Destombes disease, and Erdheim-Chester disease features and cutaneous involvement, progressing to a fatal and clonally-related acute myeloid leukemia. We reviewed the literature on similar cases and discussed the histopathological difficulties in their diagnosis and their clinical-pathological features.


Assuntos
Doença de Erdheim-Chester/genética , Histiocitose de Células de Langerhans/genética , Histiocitose Sinusal/genética , Leucemia Mieloide Aguda/patologia , MAP Quinase Quinase 1/genética , Idoso , Biópsia , Diagnóstico Diferencial , Doença de Erdheim-Chester/complicações , Doença de Erdheim-Chester/patologia , Evolução Fatal , Feminino , Histiócitos/patologia , Histiocitose de Células de Langerhans/complicações , Histiocitose de Células de Langerhans/tratamento farmacológico , Histiocitose de Células de Langerhans/patologia , Histiocitose Sinusal/complicações , Histiocitose Sinusal/patologia , Humanos , Leucemia Mieloide Aguda/diagnóstico , Leucemia Mieloide Aguda/etiologia , Masculino , Pessoa de Meia-Idade , Segunda Neoplasia Primária/patologia , Pele/patologia
18.
Dermatology ; 237(1): 1-12, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33232965

RESUMO

BACKGROUND: Coronavirus disease-19 (COVID-19) is an ongoing global pandemic caused by the "severe acute respiratory syndrome coronavirus 2" (SARS-CoV-2), which was isolated for the first time in Wuhan (China) in December 2019. Common symptoms include fever, cough, fatigue, dyspnea and hypogeusia/hyposmia. Among extrapulmonary signs associated with COVID-19, dermatological manifestations have been increasingly reported in the last few months. SUMMARY: The polymorphic nature of COVID-19-associated cutaneous manifestations led our group to propose a classification, which distinguishes the following six main clinical patterns: (i) urticarial rash, (ii) confluent erythematous/maculopapular/morbilliform rash, (iii) papulovesicular exanthem, (iv) chilblain-like acral pattern, (v) livedo reticularis/racemosa-like pattern, (vi) purpuric "vasculitic" pattern. This review summarizes the current knowledge on COVID-19-associated cutaneous manifestations, focusing on clinical features and therapeutic management of each category and attempting to give an overview of the hypothesized pathophysiological mechanisms of these conditions.


Assuntos
Acrodermatite/virologia , COVID-19/complicações , Exantema/virologia , Urticária/virologia , Acrodermatite/patologia , Exantema/patologia , Humanos , Livedo Reticular/patologia , Livedo Reticular/virologia , Gravidade do Paciente , Púrpura/patologia , Púrpura/virologia , SARS-CoV-2 , Urticária/patologia
19.
Acta Derm Venereol ; 101(11): adv00590, 2021 Nov 10.
Artigo em Inglês | MEDLINE | ID: mdl-34518893

RESUMO

The aim of this study was to detect demographic and clinical factors associated with affective symptoms and quality of life in patients with severe atopic dermatitis. First, one-way analyses of variance and correlations were performed to compare a large set of qualitative and quantitative clinical variables. Three final multivariable regression models were performed, with depression/anxiety subscales and Dermatology Life Quality Index scores as dependent variables, and the factors that were statistically significant on univariate analyses as independent ones. More severe anxiety symptoms and poorer quality of life (p < 0.01) were significantly associated with more severe depressive symptoms. Female sex and disturbed sleep (p = 0.03) were significantly associated with more severe anxiety. Finally, previous treatment with cyclosporine (p = 0.03) or methotrexate (p = 0.04), more severe depressive symptoms (p < 0.01), itch (p = 0.03), impaired sleep (p < 0.01) and perceived severity of dermatological illness (p < 0.01) were significant predictors of low quality of life. This study shows a complex interplay between the severity of atopic dermatitis, poor quality of life and presence of clinically relevant affective symptoms. These results will help dermatologists to identify patients who need psychiatric consultation within the framework of a multidisciplinary approach.


Assuntos
Dermatite Atópica , Eczema , Sintomas Afetivos , Dermatite Atópica/diagnóstico , Dermatite Atópica/epidemiologia , Feminino , Humanos , Qualidade de Vida , Índice de Gravidade de Doença
20.
Photodermatol Photoimmunol Photomed ; 37(4): 334-342, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-33458864

RESUMO

BACKGROUND: Phototherapy is a mainstay for the treatment of MF. However, there is scarce evidence for its use, mostly due to the lack of a unified schedule. AIMS: The primary aim of this study was to establish the first structured, expert-based consensus regarding the indications and technical schedules of NB-UVB and PUVA for MF. The secondary aim was to determine the consensus level for each specific item. MATERIALS & METHODS: E-delphi study. Item-specific expert consensus was defined as the number of "Totally Agree" results to ≥80% of the panelists. Cronbach alpha index ≥0.7 was used as a measure of homogeneity in the responses among questions related to the same topic. RESULTS: Overall, there was a high homogeneity among responders (0.78). On specific topics, the highest grade was observed for technical items (0.8) followed by indications for early (0.73) and advanced stages (0.7). CONCLUSIONS: Items related to the most canonical indications of phototherapy and to treatment schedules showed the highest agreements rates. There is consensus about the use of standardized treatment schedules for the induction and consolidation phases for NB-UVB and PUVA in MF.


Assuntos
Micose Fungoide , Neoplasias Cutâneas , Consenso , Técnica Delphi , Humanos , Micose Fungoide/tratamento farmacológico , Terapia PUVA , Neoplasias Cutâneas/tratamento farmacológico
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