Porokeratosis of Mibelli. A Case Report

As poroqueratoses resultam de uma hiper-proliferação clonal dos queratinócitos, encontrando-se pelo menos descritas seis formas clínicas, que partilham o achado da lamela cornóide no exame histopatológico. Os autores descrevem o caso de uma poroqueratose de Mibelli numa mulher de 27 anos, raça negra, com início na infância, eficazmente tratada com retinóide tópico, apresentando-se as manifestações típicas de uma dermatose pouco frequente e destacando-se a importância da histopatologia na confirmação do seu diagnóstico. A poroqueratose de Mibelli é uma dermatose crónica e progressiva, raramente com remissão espontânea. A sua evolução para neoplasia maligna, particularmente carcinoma espino-celular, pode ocorrer em cerca de 7% dos doentes, reforçando-se a importância de uma adequada vigilância

Cutaneous Mastocytosis in Childhood. Retrospective Study of 32 Patients

Introdução: A designação de mastocitose engloba várias entidades clinicamente distintas caracterizadas pela acumulação tissular de mastócitos. A pele é o órgão mais frequentemente envolvido. Consideram-se 4 padrões clínicos de mastocitose cutânea: urticária pigmentosa (UP), mastocitose cutânea difusa, mastocitoma e telangiectasia macularis eruptiva perstans. Na infância, a doença é habitualmente autolimitada e exclusivamente cutânea. Material e Métodos: Apresenta-se um estudo retrospetivo dos doentes com mastocitose cutânea observados na Consulta de Dermatologia Pediátrica do Hospital de Curry Cabral entre 2001 e 2010. Resultados: Foram englobados 32 doentes (20 do sexo masculino e 12 do sexo feminino). Em 90.6% dos casos, as manifestações surgiram antes dos 2 anos. Apenas foram observadas UP (53,1%) e mastocitomas (46,9%). O tronco e a raíz dos membros foram as localizações preferenciais. O sinal de Darier estava presente em 87,5% dos casos (94,1% das UP e 80% dos mastocitomas). As manifestações associadas foram: prurido (40,6%), formação de bolha (28,1%), flushing (18,8%) e dermografismo(12,5%). Não foram documentados casos de mastocitose sistémica. Dois doentes tinham história familiar de mastocitose cutânea. Não foram detetadas alterações laboratoriais significativas. Nos 16 casos determinados, os níveis séricos de Triptase-alfa foram normais. Discussão: Estes resultados estão de acordo com a literatura, nomeadamente em relação às formas clínicas mais frequentes, localizações habituais, elevada frequência do sinal de Darier e idade precoce de início. Quando determinados, os níveis séricos de Triptase-alfa foram normais, o que está de acordo com a ausência de envolvimento sistémico. A maior prevalência no sexo masculino tem sido relatada em alguns estudos

Leydig Cell Tumor of the Ovary. A Rare Case of Hyperandrogenism

Os tumores ováricos secretores de androgénios são raros constituindo cerca de 1% de todas as neoplasias do ovário. Descreve-se o caso clínico de uma doente do sexo feminino, 67 anos de idade, que recorre à consulta por queixas de hirsutismo (score 23) e alopécia com padrão androgenético A avaliação laboratorial revelou níveis séricos elevados de testosterona. A ecografia ginecológica por via transvaginal permitiu identificar a presença de um nódulo sólido no ovário esquerdo com características imagiológicas sugestivas de malignidade pelo que, a doente foi submetida a ooforectomia bilateral. O exame histopatológico revelou a presença de células de Leydig. O seguimento clínico posterior da doente permitiu constatar melhoria do quadro clínico e normalização dos níveis séricos de testosterona

Tinea Capitis in Adults. A Diagnosis to Consider?

A tinea capitis, uma infecção por fungos dermatófitos do couro cabeludo, é uma patologia rara no adulto, mesmo em áreas geográficas onde a doença é prevalente. Nestes casos está muitas vezes, mas não exclusivamente, associada a imunodepressão e habitualmente surge por exposição ao agente infeccioso em contactantes próximos. São fundamentais uma história clínica e um exame objectivo minuciosos que permitam equacionar a tinha do couro cabeludo como hipótese diagnóstica e proceder à colheita de amostras para exame micológico. Esta metodologia pode evitar a realização de exames complementares exaustivos ou a prescrição de tratamentos inadequados. Os autores descrevem três casos de tinea capitis em mulheres adultas – dois casos de infecção por Microsporum audouinii e um caso por Trichophyton soudanense. É apresentado o registo iconográfico dos casos e é efectuada breve revisão da literatura

De Novo HIV Infection Diagnoses in a Department of Dermatology and Venereology in Lisbon, Portugal

BACKGROUND: Portugal has the highest prevalence rate of HIV infection in Western Europe. The proportion of patients with a late diagnosis, carried out in full-blown AIDS stage, remains high. Skin and mucous membrane manifestations are not rare in these patients. OBJECTIVE: A demographic, clinical, and laboratorial characterization of patients with de novo HIV infection diagnosis made in the Department of Dermatology and Venereology of a central hospital in Lisbon, Portugal. METHODS: Retrospective review of medical records of adult patients newly diagnosed with HIV infection (reactive immunoassay for antibodies to HIV-1/HIV-2 or HIV p24 antigen) in the Dermatology and Venereology Department of a Portuguese central hospital in the period between January 2005 and December 2013. RESULTS: During the study period, 97 new cases were diagnosed, 70 men and 27 women. The median age at diagnosis was 36 years. Of the total, 50 cases were diagnosed with a concomitant sexually transmitted infection (STI), more frequently syphilis and ano-genital HPV infection. The remaining 47 patients were diagnosed with other dermatological conditions such as prurigo nodularis, psoriasis, and Kaposi's sarcoma. The duration of complaints that lead to medical attention ranged from <1 week to 8 years, being significantly lower in patients diagnosed with a concomitant STI (p < 0.01). Basal viral load was also lower in this group of patients (p < 0.05). Of all the new diagnosed cases, 80% of patients are currently retained in care. CONCLUSION: This study grants a descriptive overview of de novo HIV infection diagnoses performed by dermatovenereologists in a central hospital in Lisbon, Portugal. As in the past, the importance of Dermato-Venereology for HIV infection diagnosis remains present in daily clinical practice

Updates on post-release mortality of shortfin mako in the Atlantic using satellite telemetry

This paper provides an update of the study on post-release mortality of the shortfin mako, Isurus oxyrinchus developed within the ICCAT Shark Research and Data Collection Program (SRDCP). Up to date, 43 tags (14 sPATs and 29 miniPATs) have been deployed by observers on Brazilian, Portuguese, Spanish, Uruguayan, and US vessels in the temperate NE and NW, Equatorial and SW Atlantic. Data from 35 out of 43 tagged specimens could be used to obtain preliminary information regarding post-release mortality, resulting in a total of 8 mortality and 27 survival events.This study was carried out as part of a cooperative work conducted by the ICCAT Shark species group integrated in the ICCAT Shark Research and Data Collection Program (SRDCP). The authors are grateful to all fishery observers and longline skippers from the Nations involved in this study. Tags from additional sources have been contributed and deployed with several national Projects, specifically: Project "LL-Sharks: Mitigação das capturas de tubarões na pescaria de palangre de superfície (Ref: 31-03-05-FEP-44, funded by PROMAR)", Project "MAKO-WIDE - "A wide scale inter-hemispheric and inter-disciplinary study aiming the conservation of the shortfin mako shark in the Atlantic Ocean (Ref: FAPESP/19740/2014)", funded by FCT (Portuguese Foundation for Science and Technology) and FAPESP (São Paulo Research Foundation, Brazil), and Project SAFEWATERS SC7 (The provision of advice on the conservation of pelagic sharks associated to fishing activity under EU Sustainable Fisheries Partnership Agreements in the Atlantic Ocean) under the Framework Contract MARE/2012/21, funded by the European Commission. Additional satellite tags were acquired by NOAA in US-Uruguay and US-Portugal-Uruguay collaboration initiatives. Rui Coelho is supported by an Investigador-FCT contract from the Portuguese Foundation for Science and Technology (FCT) supported by the EU European Social Fund and the Programa Operacional Potencial Humano (Ref: IF/00253/2014). Catarina C. Santos is supported by an FCT Doctoral grant (Ref: SFRH/BD/139187/2018).info:eu-repo/semantics/draf

Sensitivity of the Cherenkov Telescope Array to TeV photon emission from the Large Magellanic Cloud

A deep survey of the Large Magellanic Cloud at ∼0.1-100 TeV photon energies with the Cherenkov Telescope Array is planned. We assess the detection prospects based on a model for the emission of the galaxy, comprising the four known TeV emitters, mock populations of sources, and interstellar emission on galactic scales. We also assess the detectability of 30 Doradus and SN 1987A, and the constraints that can be derived on the nature of dark matter. The survey will allow for fine spectral studies of N 157B, N 132D, LMC P3, and 30 Doradus C, and half a dozen other sources should be revealed, mainly pulsar-powered objects. The remnant from SN 1987A could be detected if it produces cosmic-ray nuclei with a flat power-law spectrum at high energies, or with a steeper index 2.3-2.4 pending a flux increase by a factor of &gt;3-4 over ∼2015-2035. Large-scale interstellar emission remains mostly out of reach of the survey if its &gt;10 GeV spectrum has a soft photon index ∼2.7, but degree-scale 0.1-10 TeV pion-decay emission could be detected if the cosmic-ray spectrum hardens above &gt;100 GeV. The 30 Doradus star-forming region is detectable if acceleration efficiency is on the order of 1−10 per cent of the mechanical luminosity and diffusion is suppressed by two orders of magnitude within &lt;100 pc. Finally, the survey could probe the canonical velocity-averaged cross-section for self-annihilation of weakly interacting massive particles for cuspy Navarro-Frenk-White profiles

Sensitivity of the Cherenkov Telescope Array to spectral signatures of hadronic PeVatrons with application to Galactic Supernova Remnants

The local Cosmic Ray (CR) energy spectrum exhibits a spectral softening at energies around 3~PeV. Sources which are capable of accelerating hadrons to such energies are called hadronic PeVatrons. However, hadronic PeVatrons have not yet been firmly identified within the Galaxy. Several source classes, including Galactic Supernova Remnants (SNRs), have been proposed as PeVatron candidates. The potential to search for hadronic PeVatrons with the Cherenkov Telescope Array (CTA) is assessed. The focus is on the usage of very high energy $\gamma$-ray spectral signatures for the identification of PeVatrons. Assuming that SNRs can accelerate CRs up to knee energies, the number of Galactic SNRs which can be identified as PeVatrons with CTA is estimated within a model for the evolution of SNRs. Additionally, the potential of a follow-up observation strategy under moonlight conditions for PeVatron searches is investigated. Statistical methods for the identification of PeVatrons are introduced, and realistic Monte--Carlo simulations of the response of the CTA observatory to the emission spectra from hadronic PeVatrons are performed. Based on simulations of a simplified model for the evolution for SNRs, the detection of a $\gamma$-ray signal from in average 9 Galactic PeVatron SNRs is expected to result from the scan of the Galactic plane with CTA after 10 hours of exposure. CTA is also shown to have excellent potential to confirm these sources as PeVatrons in deep observations with $\mathcal{O}(100)$ hours of exposure per source.Comment: 34 pages, 16 figures, Accepted for publication in Astroparticle Physic

Identification and reconstruction of low-energy electrons in the ProtoDUNE-SP detector

Measurements of electrons from $\nu_e$ interactions are crucial for the Deep Underground Neutrino Experiment (DUNE) neutrino oscillation program, as well as searches for physics beyond the standard model, supernova neutrino detection, and solar neutrino measurements. This article describes the selection and reconstruction of low-energy (Michel) electrons in the ProtoDUNE-SP detector. ProtoDUNE-SP is one of the prototypes for the DUNE far detector, built and operated at CERN as a charged particle test beam experiment. A sample of low-energy electrons produced by the decay of cosmic muons is selected with a purity of 95%. This sample is used to calibrate the low-energy electron energy scale with two techniques. An electron energy calibration based on a cosmic ray muon sample uses calibration constants derived from measured and simulated cosmic ray muon events. Another calibration technique makes use of the theoretically well-understood Michel electron energy spectrum to convert reconstructed charge to electron energy. In addition, the effects of detector response to low-energy electron energy scale and its resolution including readout electronics threshold effects are quantified. Finally, the relation between the theoretical and reconstructed low-energy electron energy spectrum is derived and the energy resolution is characterized. The low-energy electron selection presented here accounts for about 75% of the total electron deposited energy. After the addition of lost energy using a Monte Carlo simulation, the energy resolution improves from about 40% to 25% at 50~MeV. These results are used to validate the expected capabilities of the DUNE far detector to reconstruct low-energy electrons.Comment: 19 pages, 10 figure