Sweet's Syndrome and Relapsing Polychondritis Signal Myelodysplastic Syndrome

Certas dermatoses, pertencentes ao grupo das síndromes paraneoplásicas mucocutâneas, podem ser o prenúncio de uma neoplasia previamente não conhecida. Tanto a síndrome de Sweet como a policondrite recidivante incluem-se neste grupo. A síndrome de Sweet e a PR são raramente encontradas em um mesmo paciente. A presença de policondrite recidivante e síndrome de Sweet em um mesmo paciente tem se revelado mais frequente em pacientes com neoplasias associadas, sobretudo hematológicas. Relata-se o caso de paciente do sexo masculino, 79 anos, com síndrome de Sweet e policondrite recidivante, em quem, subsequentemente, foi diagnosticada uma síndrome mielodisplásica. Palavras-chave: Policondrite recidivante; Síndrome de Sweet; Síndromes paraneoplásica

The medico-legal importance of establishing human identity by palatal rugoscopy: evaluation of the immutability and individuality of palatal rugae under the influence of ante mortem orthodontic treatment

This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited."Introduction: The palatal rugae can be an alternative method of forensic identification. Through the years, several investigations focused on the effect of orthodontic treatment in the palatal rugae pattern. Objectives: Evaluate the concepts of immutability and individuality of the palatal rugae in a Portuguese adult population submitted to orthodontic treatment, for the purpose of medico-legal identification through Thomas and Kotze classification system. Additionally, we wanted to establish comparison of the palatal rugae of each subject, and between genders. Materials and Methods: Thirty three pairs of study dental casts, from thirty three patients submitted to orthodontic treatment in the Department of Orthodontics in College of Dentistry - University of Lisbon, were photographed and classified according to the classification system described by Thomas and Kotze. We proceeded to a statistical analysis running SPSS for Windows, version 20.0, using descriptive analysis and tests, with an inclusion level p <0.05. The tests applied were normality tests and T Student for paired samples. Results: The number and length of primary rugae remain identical when comparing the situation before and after orthodontic treatment. The number of secondary rugae decreases after orthodontic treatment. The variation of the angle of divergence was not statistically significant. The area of primary rugae presented statistically significant reduction after orthodontic treatment. There has no statistically significant differences between genders for total number number of rugae or average length of primary rugae (p <0.05). Discussion and Conclusion: The palatal rugae pattern does not remain stable after orthodontic treatment, and this refutes the supposed long term stability of the palatal rugae pattern. Therefore, it influences the ability to establish a positive medico-legal identification of a recent copse, if the person was submitted to ante mortem orthodontic treatment. The identification might still be possible if we possess a last ante mortem palatal record in these situations, to allow identification based on positive individual characteristics, through comparison with the post-mortem record. Considering gender, no statistically significant differences were found. This subject remains controversial and deserves further research.

The Tip of the Iceberg: Cutaneous Manifestations of Paediatric Diseases with Neurological Involvement

Introdução: Algumas alterações cutâneas podem ser as primeiras manifestações clínicas de diversas entidades nosológicas com atingimento sistémico. O presente trabalho tem como objectivo rever a semiologia dermatológica relevante no contexto das doenças com envolvimento neurológico na infância. Material e Métodos: Revisão dos artigos indexados à MedLine publicados nos últimos 12 anos e com relevância para o tema. Resultados: Os principais grupos nosológicos relevantes para o tema compreendem as genodermatoses (com destaque para as síndromes neurocutâneas), as alterações da pigmentação, as doenças vasculares, as endócrinas, os défices enzimáticos congénitos e os disrafismos espinhais ocultos. Discussão: O reconhecimento da semiologia cutânea específica é importante pois pode permitir um diagnóstico muito mais precoce

Switching Biologics in Severe Pediatric Psoriasis: a Retrospective Analysis

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High-temperature corrosion performance of austenitic stainless steels type AISI 316L and AISI 321H, in molten solar salt

ABSTRACT: The corrosion rates of AISI 316L and AISI 321H austenitic stainless steel, immersed in a stagnant isothermal mixture of 60% NaNO3 and 40% KNO3 molten salt at 550 degrees C in atmospheric air are 8.6 and 9.0 mu m/yr, respectively. The corrosion mechanism was proposed by recording the weight changes of the steel coupons at different time intervals up to 3000 h, and by the characterization of multilayer oxide scales formed on the steel surface. Multilayers made of different oxides, mainly Fe2O3 and Fe3O4, are the principal scale products. At 3000 h, the thickness of the scale layer formed on AISI 321H (7.5 +/- 2.9 mu m) is slightly higher than the one formed at the AISI 316L (6.9 +/- 2.1 mu m). This small difference might reflect the partial spallation of the corrosion layer on AISI 321H, which is seen for times longer than 1000 h. A minimal change of the composition of the molten nitrate salt is observed in time and is predominantly due to the appearance of soluble chromate products and nitrite compounds (0.004 wt% and 1.4 wt% at 3000 h, respectively). The observed corrosion behaviour of these alloys shows that they are good candidate for usage as containers of molten nitrate salts in the thermal energy storage (TES) system for a CSP plant.info:eu-repo/semantics/publishedVersio

Transformation of a Cutaneous Follicle Center Lymphoma to a Diffuse Large B-Cell Lymphoma—An Unusual Presentation

Primary cutaneous follicle center lymphoma (PCFCL) is characterized by a proliferation of follicle center cells in the skin. A definitive diagnosis is frequently delayed because of difficulties in interpretation of the histopathologic findings. It has an excellent prognosis with a 5-year survival over 95% and its risk of transformation has not been established. We describe a case report of man with a gastric diffuse large B-cell lymphoma (DLBCL) referred to our clinic because of nodules in the back that had gradually developed over a period of 10 years. A biopsy performed 3 years before was interpreted as reactive follicular hyperplasia. A new skin biopsy revealed a diffuse large B-cell lymphoma and immunoglobulin heavy chain gene rearrangements from the initial skin biopsy (PCBCL) and the DLBCL gastric biopsy were studied by polymerase chain reaction and an identical clonal rearrangement was detected which was highly suggestive of a transformation lymphoma

Erythrodermia: the First Manifestation of Combined Immunodeficiency

A Doença do Enxerto Contra o Hospedeiro associada a transfusão (DEH-AT) é uma complicação rara da transfusão de hemoderivados não irradiados que afecta habitualmente indivíduos pertencentes a grupo de risco, nomeadamente crianças com imunodeficiência congénita. Caracteriza-se por um envolvimento cutâneo, gastrointestinal e hematológico, sendo habitualmente fatal. Descreve-se um caso DEH-AT atenuada que constituiu o indício para o diagnóstico de uma imunodeficiência combinada. A doença manifestou-se com rash característico mas não apresentou envolvimento digestivo ou hematológico, provavelmente pela corticoterapia concomitante. Tal facto permitiu a sobrevivência da criança e o diagnóstico de uma imunodeficência combinada. O diagnóstico definitivo de DEH-AT pode ser difícil uma vez que as manifestações clínicas e histológicas mimetizam, com frequência, outras situações, tais como toxidermia ou doença viral.Transfusion-associated graft-versus-host disease (TA-GVHD) is a rare complication of transfusion of nonirradiated blood components. It usually affects children in high-risk groups, including those who have primary immunode ficiencies (PIDs). It usually presents with skin, hepatic, digestive, and hematologic involvement and is normally fatal. We report a case of a nonlethal, attenuated, TA-GVHD which was the clue to the diagnosis of combined immunodeficiency. The disease was marked by the presence of a severe rash but lacked all the other usual manifestations and wasn’t fatal due to the fact that this child was under high-dose corticotherapy. This led to the survival of this child and allowed the diagnosis of a combined immunodeficiency. The definitive diagnosis of GVHD can be problematic because the clinical and histological features can mimic other conditions such as drug eruptions, viral rash or eczema.info:eu-repo/semantics/publishedVersio