McCune-Albright syndrome - A case report with transmission electron microscopy

Abstract McCune - Albright syndrome is a genetic disease with cutaneous mosaicism caused by post-zygotic activating mutations in GNAS locus, it has a triad of fibrous bone dysplasia, café-au-lait macules and precocious puberty. We examined a 22-year-old female patient with café au lait spot in right side of the abdomen, with a chessboard - like distribution, extending to right thigh with geographical contours, she has also an ovarian cyst, scoliosis and truncal obesity. Biopsies were taken from the hyperpigmented area and processed for light microscopy and for transmission electron microscopy. Light microscopy showed increased melanin pigment with HE staining. Immunohistochemistry with melanocytic markers (HMB-45 and Melan-A) revealed a normal number of melanocytes. Transmission electron microscopy demonstrated normal epidermal structures, such as desmosomes, cytokeratin filaments and hemidesmosomes. With high magnifications an irregular melanossomal contour was seen, with some indentations in their outline.

McCune-Albright syndrome - A case report with transmission electron microscopy.

McCune - Albright syndrome is a genetic disease with cutaneous mosaicism caused by post-zygotic activating mutations in GNAS locus, it has a triad of fibrous bone dysplasia, café-au-lait macules and precocious puberty. We examined a 22-year-old female patient with café au lait spot in right side of the abdomen, with a chessboard - like distribution, extending to right thigh with geographical contours, she has also an ovarian cyst, scoliosis and truncal obesity. Biopsies were taken from the hyperpigmented area and processed for light microscopy and for transmission electron microscopy. Light microscopy showed increased melanin pigment with HE staining. Immunohistochemistry with melanocytic markers (HMB-45 and Melan-A) revealed a normal number of melanocytes. Transmission electron microscopy demonstrated normal epidermal structures, such as desmosomes, cytokeratin filaments and hemidesmosomes. With high magnifications an irregular melanossomal contour was seen, with some indentations in their outline.

Transmission and scanning electron microscopy of cutis rhomboidalis

Abstract Cutis rhomboidalis nuchae was assessed in a 65-year-old patient. Optical microscopy showed basophilic agglomerations in the reticular dermis with decreased elastic fibers. Trans- mission electron microscopy showed elongated, curved and fragmented structures, and in their interior the presence of electron-dense lumps was reduced and irregular, similar to modified elastic fibers, whereas the collagen fibers had a normal aspect. Scanning electron microscopy showed deposits between the bundles of collagen, resembling pebbles or stones. These findings demonstrate that, at one stage of the disease, the collagen remains normal and the alterations are seen in the elastic tissue.

Transmission and scanning electron microscopy of cutis rhomboidalis.

Cutis rhomboidalis nuchae was assessed in a 65-year-old patient. Optical microscopy showed basophilic agglomerations in the reticular dermis with decreased elastic fibers. Transmission electron microscopy showed elongated, curved and fragmented structures, and in their interior the presence of electron-dense lumps was reduced and irregular, similar to modified elastic fibers, whereas the collagen fibers had a normal aspect. Scanning electron microscopy showed deposits between the bundles of collagen, resembling pebbles or stones. These findings demonstrate that, at one stage of the disease, the collagen remains normal and the alterations are seen in the elastic tissue.

Persistent Symptoms and Disability After COVID-19 Hospitalization: Data From a Comprehensive Telerehabilitation Program.

OBJECTIVE: To report symptoms, disability, and rehabilitation referral rates after coronavirus disease 2019 (COVID-19) hospitalization in a large, predominantly older population. DESIGN: Cross-sectional study, with postdischarge telemonitoring of individuals hospitalized with confirmed COVID-19 at the first month after hospital discharge, as part of a comprehensive telerehabilitation program. SETTING: Private verticalized health care network specialized in the older population. PARTICIPANTS: Individuals hospitalized because of COVID-19. We included 1696 consecutive patients, aged 71.8±13.0 years old and 56.1% female. Comorbidities were present in 82.3% of the cases (N=1696). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Dependence for basic activities of daily living (ADL) and instrumental activities of daily living (IADL) using the Barthel Index and Lawton's Scale. We compared the outcomes between participants admitted to the intensive care unit (ICU) vs those admitted to the ward. RESULTS: Participant were followed up for 21.8±11.7 days after discharge. During postdischarge assessment, independence for ADL was found to be lower in the group admitted to the ICU than the ward group (61.1% [95% confidence interval (CI), 55.8%-66.2%] vs 72.7% [95% CI, 70.3%-75.1%], P<.001). Dependence for IADL was also more frequent in the ICU group (84.6% [95% CI, 80.4%-88.2%] vs 74.5%, [95% CI, 72.0%-76.8%], P<.001). Individuals admitted to ICU required more oxygen therapy (25.5% vs 12.6%, P<.001), presented more shortness of breath during routine (45.2% vs 34.5%, P<.001) and nonroutine activities (66.3% vs 48.2%, P<.001), and had more difficulty standing up for 10 minutes (49.3% vs 37.9% P<.001). The rehabilitation treatment plan consisted mostly of exercise booklets, which were offered to 65.5% of participants. The most referred rehabilitation professionals were psychologists (11.8%), physical therapists (8.0%), dietitians (6.8%), and speech-language pathologists (4.6%). CONCLUSIONS: Individuals hospitalized because of COVID-19 present high levels of disability, dyspnea, dysphagia, and dependence for both ADL and IADL. Those admitted to the ICU presented more advanced disability parameters.

Perioperative Immunonutrition in Elderly Patients Undergoing Total Hip and Knee Arthroplasty: Impact on Postoperative Outcomes.

BACKGROUND: Arthroplasties in elderly patients are surgeries performed to ensure their quality of life. Perioperative care with specific nutrients can improve nutrition status and metabolic response to orthopedic surgeries, such as total knee arthroplasty (TKA) and total hip arthroplasty (THA). METHODS: Retrospective study with elderly patients divided into 2 groups: control and immunonutrition. The immunonutrition group was instructed to start oral intake of the nutrition supplement 5 days before and to resume it 5 days after arthroplasty (200 mL, 3 times per day). The following were analyzed as primary and secondary outcomes: length of stay (LOS), infectious and noninfectious complications, need for intensive care unit (ICU), transfusion requirement, and C-reactive protein. RESULTS: A total of 3015 elderly patients met the inclusion criteria: control group (n = 1398) and immunonutrition group (n = 1617). Overall, 81.2% were women and mean age was 72.6 ± 6.9 years. Immunonutrition group had a shorter LOS in hours (32.0 ± 19.4 vs 56.0 ± 26.4; P < .001) and lower rates of infectious complications (2.2% vs 4.6%; P < .001). Noninfectious complications and need for ICU also had lower rates in the immunonutrition group. In the logistic regression analysis, immunonutrition reduced the chance of infectious complications by 55% (odds ratio [OR], 0.45; 95% CI, 0.30-0.68; P < .001) even after adjusting for variables (OR, 0.45; 95% CI, 0.28-0.71; P < .001). CONCLUSION: Perioperative immunonutrition in elderly patients undergoing THA or TKA may shorten postoperative LOS and reduce infectious and noninfectious complications and transfusion requirement.

Sclerosing variant of well-differentiated testicular liposarcoma: a case report / Lipossarcoma testicular bem diferenciado com variante esclerosante: um relato de caso

ABSTRACT Testicular cancers are classified in germ cell and non-germ cell tumors, as well as, liposarcomas. We report the case of a patient with a large testicular liposarcoma, submitted to surgical treatment with excision of scrotal pouch and segment of the spermatic cord, and the left testicle, showing a good evolution. This report presents one of the first cases of a sclerosing variant of well-differentiated testicular liposarcoma, large in size and with no association with another cancer. Due to their location, the diagnosis is difficult and unusual. Complete tumor resection and regular medical follow-up show a good prognosis, less recurrence, and little cellular differentiation.

RESUMEN Los cánceres de testículo se clasifican en tumores de células germinales y células no germinales, así como en liposarcomas. Presentamos el caso de un paciente con un gran liposarcoma testicular, sometido a tratamiento quirúrgico con exéresis de la bolsa escrotal y segmento de cordón espermático y testículo izquierdo, con buena evolución. Este informe presenta uno de los primeros casos de una variante esclerosante de liposarcoma testicular bien diferenciado, de gran tamaño y sin asociación con otro cáncer. Debido a su ubicación, el diagnóstico es difícil e inusual. La resección completa del tumor y el seguimiento médico regular muestran un buen pronóstico, menor recidiva, y poca diferenciación celular.

RESUMO Os tumores testiculares são classificados em células germinativas e não germinativas, assim como os lipossarcomas. Relatamos o caso de um paciente com lipossarcoma testicular de grande dimensão submetido a tratamentos cirúrgicos com ressecção de bolsa escrotal e segmento do cordão espermático e do testículo esquerdo, apresentando boa evolução do quadro. O relato traz um dos primeiros casos de lipossarcoma testicular bem diferenciado com variante esclerosante, de grande dimensão e sem associação a outra neoplasia. Devido à localização, apresenta diagnóstico difícil e pouco habitual. Com a ressecção total do tumor e o acompanhamento médico regular, o paciente apresenta bom prognóstico, menor recidiva e pouca diferenciação celular.

Light and electron microscopy of chlorpromazine-induced hyperpigmentation.

Chlorpromazine may induce abnormal skin hyperpigmentation in exposed areas, described as slate-gray, purple, or blue-grayish discoloration. A 58-year-old man with schizophrenia, had been taking chlorpromazine for 5 years, and his sun-exposed skin areas exhibited a blue-grayish color. Large deposits of brown pigment and granular basophilic material were seen in the dermis with light microscopy. HMB-45 and anti-Melan-A antibody immunostaining labeled some pigment in the dermis. Transmission electron microscopy identified deposits among dermal collagen bundles collagen in both transverse and longitudinal sections. In the latter, an arboriform aspect of deposits was quite clear, and some melanophages were also seen. The three-dimensional examination of the dermis with scanning electron microscopy also identified deposits, which at higher magnification demonstrated an appearance in the shape of leaves, grass-like, interspersed with normal collagen. These results suggest a complex pathogenic mechanism, including deposition of dermal melanin together with drug itself and potentially additional unknown metabolites.

Scanning electron microscopy of dermatofibroma.

Dermatofibroma is a proliferation of spindle cells located in the dermis. We used scanning electron microscopy to examine two histologically confirmed lesions and observed preserved collagen bundles in the perilesional area. In the lesional area, the collagen was denser, without formation of bundles. Higher magnification showed collagen with mesh-like appearance similar to stretched tufts of cotton. Very high magnification evidenced the tufts of cotton and spindle cells measuring 2 to 12 microns.

Nodular fasciitis: case report / Fasciíte nodular: relato de caso

ABSTRACT Nodular fasciitis (NF) is a rare fibroblast proliferation of unknown etiology, with benign, rapid clonal growth, from a superficial fascia to the subcutaneous tissue or an adjacent muscular layer. Also known as pseudosarcomatous fasciitis, this clinical syndrome is characterized as a solitary mass of hardened consistency, painless palpation and no gender preference. The definitive diagnosis is made by immunohistochemistry and surgery is the treatment of choice.

RESUMEN La fascitis nodular (FN) es una lesión proliferativa fibroblástica rara, de etiología desconocida, naturaleza benigna, crecimiento rápido y probablemente clonal, que se origina en una fascia superficial hacia el tejido subcutáneo o una camada muscular adyacente. También conocida como fascitis pseudosarcomatosa, este síndrome clínico se caracteriza por un tumor solitario de consistencia endurecida, ligero dolor a la palpación y sin predilección por sexo. Su diagnóstico definitivo se hace por inmunohistoquímica, y el tratamiento de elección es el quirúrgico.

RESUMO A fasciíte nodular (FN) é uma lesão proliferativa fibroblástica rara, de etiologia desconhecida, caráter benigno, crescimento rápido e provavelmente clonal, que se origina a partir de uma fáscia superficial para o tecido subcutâneo ou uma camada muscular adjacente. Também conhecida como fasciíte pseudossarcomatosa, esta síndrome clínica caracteriza-se por uma massa solitária de consistência endurecida, pouco dolorosa à palpação e sem predileção por gênero. O diagnóstico definitivo é feito por imuno-histoquímica, e o tratamento de escolha é o cirúrgico.

Scanning electron microscopy of dermatofibroma

Abstract: Dermatofibroma is a proliferation of spindle cells located in the dermis. We used scanning electron microscopy to examine two histologically confirmed lesions and observed preserved collagen bundles in the perilesional area. In the lesional area, the collagen was denser, without formation of bundles. Higher magnification showed collagen with mesh-like appearance similar to stretched tufts of cotton. Very high magnification evidenced the tufts of cotton and spindle cells measuring 2 to 12 microns.

Ultrastructural aspects of pseudoxanthoma elasticum.

We report the ultrastructural findings in a case of a 72-year-old black woman with confluent yellowish papules in the cervical region. She had no comorbidities. Ophthalmological examination, electrocardiogram, and echocardiogram were normal. Hematoxylin-eosin staining of the affected skin showed strong alterations in the mid-dermis with irregular clumps of eosinophilic material and loss of the normal parallel arrangement of collagen bundles. Orcein staining revealed that the elastic fibers lost their normal linear configuration, showing clump fragmentation, sometimes forming square structures. Transmission electron microscopy showed aberrant elastic fibers with an irregular outline and heterogenic inner structures. We also observed small elastic fibers. Collagen fibers showed a normal structure with irregular distribution. Scanning electron microscopy revealed important disorganization of collagen fibers and small stone-like deposits measuring around 5 µm associated with bigger structures ranging from 10-16 µm. Higher magnification revealed that these small stone-like structures were sometimes polyhedral-shaped or squared.

Ultrastructural aspects of pseudoxanthoma elasticum

Abstract: We report the ultrastructural findings in a case of a 72-year-old black woman with confluent yellowish papules in the cervical region. She had no comorbidities. Ophthalmological examination, electrocardiogram, and echocardiogram were normal. Hematoxylin-eosin staining of the affected skin showed strong alterations in the mid-dermis with irregular clumps of eosinophilic material and loss of the normal parallel arrangement of collagen bundles. Orcein staining revealed that the elastic fibers lost their normal linear configuration, showing clump fragmentation, sometimes forming square structures. Transmission electron microscopy showed aberrant elastic fibers with an irregular outline and heterogenic inner structures. We also observed small elastic fibers. Collagen fibers showed a normal structure with irregular distribution. Scanning electron microscopy revealed important disorganization of collagen fibers and small stone-like deposits measuring around 5 µm associated with bigger structures ranging from 10-16 µm. Higher magnification revealed that these small stone-like structures were sometimes polyhedral-shaped or squared.

Meningioma cervical intramedular: um relato de caso raro / Intramedullary Cervical Spinal Cord Meningioma: A Rare Case Report

O meningioma intramedular é uma entidade relatada clinicamente de forma rara, tendo apenas nove relatos. Neste estudo, descrevemos o caso de um paciente masculino, 67 anos, com evolução de 25 anos, severos déficits motores, sensitivos e autonômicos. O diagnóstico foi confirmado por ressonância magnética mostrando uma massa intramedular central ao nível de C6-C7. O paciente foi submetido à microneurocirurgia com monitoração neurofisiológica, obtendo-se ressecção total e em bloco da lesão tumoral. Os achados histopatológicos e imuno-histoquímicos confirmaram meningioma angiomatoso com expressão multifocal de GFAP e de neurofilamento. O transoperatório ocorreu sem intercorrências, mas, quinze dias após, o paciente evoluiu com piora motora à esquerda. Apesar de extremamente raros, os meningiomas devem ser considerados um diagnóstico possível de tumoração intramedular.

Intramedullary meningioma is a rarely reported clinical entity, with only 9 cases reported to date. We describe a male patient, 67 years old, with 25 years of evolution and severe motor, sensory, and autonomic deficits. Preoperative magnetic resonance imaging (MRI) showed an intramedullary mass at the C6-C7 level. The patient underwent microneurosurgery and neurophysiological monitoring confirmed total in bloc resection. The histopathological and immunohistochemical findings confirmed angiomatous meningioma with multifocal expression of glial fibrillary acidic protein (GFAP) and neurofilament. Trans-operative was uneventful and, fifteen days later, he Costa et al.

Paracoccidioidomycosis in southern Rio Grande do Sul: a retrospective study of histopathologically diagnosed cases.

Paracoccidioidomycosis (PCM) is a systemic mycosis caused by the fungus Paracoccidioides brasiliensis and is endemic to Brazil. The aim of this study was to perform a retrospective analysis of the PCM cases in the countryside south of Rio Grande do Sul, Brazil. The files from four histopathology laboratories located in the city of Pelotas were obtained, and all of the epidemiological and clinical data from the PCM diagnosed cases were collected for analysis. A total of 123 PCM cases diagnosed between 1966 and 2009 were selected. Of these patients, 104 (84.5%) were male, and 17 were female. The patients ranged from 02 to 92 years of age. Fifty-two cases (41.9%) were obtained from the oral pathology laboratory, and the remaining 71 cases (58.1%) were obtained from the three general pathology laboratories. Of all of the patients studied, 65.2% lived in rural zones and worked in agriculture or other related fields. Data on the evolution of this disease was available for 43 cases, and the time frame ranged from 20 to 2920 days (mean = 572.3 days). An accurate diagnosis performed in less than 30 days only occurred in 21% of the cases. PCM is endemic to the countryside of Rio Grande do Sul. Therefore, it is recommended that PCM be included as a differential diagnosis, mainly for individuals between 30 and 60 years of age, living in rural zones and who have respiratory signs and associated-oropharyngeal lesions.

Paracoccidioidomycosis in southern Rio Grande do Sul: a retrospective study of histopathologically diagnosed cases

Paracoccidioidomycosis (PCM) is a systemic mycosis caused by the fungus Paracoccidioides brasiliensis and is endemic to Brazil. The aim of this study was to perform a retrospective analysis of the PCM cases in the countryside south of Rio Grande do Sul, Brazil. The files from four histopathology laboratories located in the city of Pelotas were obtained, and all of the epidemiological and clinical data from the PCM diagnosed cases were collected for analysis. A total of 123 PCM cases diagnosed between 1966 and 2009 were selected. Of these patients, 104 (84.5%) were male, and 17 were female. The patients ranged from 02 to 92 years of age. Fifty-two cases (41.9%) were obtained from the oral pathology laboratory, and the remaining 71 cases (58.1%) were obtained from the three general pathology laboratories. Of all of the patients studied, 65.2% lived in rural zones and worked in agriculture or other related fields. Data on the evolution of this disease was available for 43 cases, and the time frame ranged from 20 to 2920 days (mean = 572.3 days). An accurate diagnosis performed in less than 30 days only occurred in 21% of the cases. PCM is endemic to the countryside of Rio Grande do Sul. Therefore, it is recommended that PCM be included as a differential diagnosis, mainly for individuals between 30 and 60 years of age, living in rural zones and who have respiratory signs and associated-oropharyngeal lesions.

Extensive hydroa vacciniforme.

Hydroa Vaciniforme is a very rare photodermatosis that is mainly seen in childhood. An 18 year old female student reported that since the age of 5 she has been suffering necrotic lesions and vesicles lesions in exposed areas, leaving asymptomatic varioliform scars, which worsened in summer. Light microscopy showed epidermal necrosis with lymphocytic infiltration . Sunscreens were prescribed with light improvement.

Extensive hydroa vacciniforme / Hidroa vaciniforme extensa

Hydroa Vaciniforme is a very rare photodermatosis that is mainly seen in childhood. An 18 year old female student reported that since the age of 5 she has been suffering necrotic lesions and vesicles lesions in exposed areas, leaving asymptomatic varioliform scars, which worsened in summer. Light microscopy showed epidermal necrosis with lymphocytic infiltration . Sunscreens were prescribed with light improvement.

O Hidroa Vaciniforme é uma fotodermatose muito rara vista geralmente na infância. Uma paciente de 18 anos foi examinada, a qual apresenta lesões vesiculosas e necróticas varioliformes nas áreas fotoexpostas, que evoluem para cicatrizes atróficas, piorando no verão. A microscopia óptica mostrou necrose epidérmica com infiltrado linfocítico. Houve pouca melhora com uso de filtros solares.