Enhancing understanding of SARS-CoV-2 infection among individuals with Down syndrome: An integrative review

ABSTRACT BACKGROUND: Down syndrome (DS) is a non-rare genetic condition that affects approximately 1 in every 800 live births worldwide. Further, it is associated with comorbidities, anatomical alterations of the respiratory tract, and immunological dysfunctions that make individuals more susceptible to respiratory infections. OBJECTIVE: To systematize the current scientific knowledge about the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection among individuals with DS. DESIGN AND SETTING: This integrative review was conducted at the Universidade Federal de São Carlos, São Paulo, Brazil. METHODS: This review was conducted in the following databases: the Virtual Health Library (Biblioteca Virtual em Saúde, BVS), PubMed, and Web of Science, using MeSH descriptors. The search included English or Portuguese studies published between January 1, 2020, and October 14, 2022. RESULTS: A total of 55 articles from 24 countries were selected, comprising 21 case-control or cohort studies, 23 case reports or series, and 11 narrative reviews or opinion studies. The articles were grouped into five categories: previous comorbidities, coronavirus disease 2019 (COVID-19) clinical features and evolution, cytokine storm and interleukins, living in institutions as a risk factor, and behavioral actions as a protective factor against SARS-CoV-2 infection. CONCLUSION: Individuals with DS are more susceptible to COVID-19 infection due to variables such as previous comorbidities, immunological factors, and their habitable environments. These aspects confer a higher risk of infection and an unfavorable clinical course. The precise pathways involved in the pathophysiology of COVID-19 in individuals with DS are not clear, thus requiring further studies. SYSTEMATIC REVIEW REGISTRATION: The Open Science Framework registered the research protocol (https://osf.io/jyb97/).

Scientific Publications on Thyroid Ultrasound between 2001 and 2020: Differences in Research Characteristics by Disciplines

Objective@#To analyze the characteristics and trends of scientific publications on thyroid ultrasound (US) from 2001 to 2020, specifically examining the differences among disciplines. @*Materials and Methods@#The MEDLINE database was searched for scientific articles on thyroid US published between 2001 and 2020 using the PubMed online service. The evaluated parameters included year of publication, type of document, topic, funding, first author’s specialty, journal name, subject category, impact factor, and quartile ranking of the publishing journal, country, and language. Relationships between the first author’s specialty (radiology, internal medicine, surgery, otorhinolaryngology, and miscellaneous) and other parameters were analyzed. @*Results@#A total of 2917 thyroid US publications were published between 2001 and 2020, which followed an exponential growth pattern, with an annual growth rate of 11.6%. Radiology produced the most publications (n = 1290, 44.2%), followed by internal medicine (n = 716, 24.5%), surgery (n = 409, 14.0%), and otorhinolaryngology (n = 171, 5.9%). Otorhinolaryngology and internal medicine published significantly more case reports than radiology (p < 0.001, each). Radiology published a significantly higher proportion of publications on imaging diagnosis (p < 0.001 for all) and a significantly lower proportion of publications on biopsy (p < 0.001 for all) than the other disciplines. Publications produced by radiology authors were less frequently published in Q1 journals than those from other disciplines (p < 0.005 for internal medicine and miscellaneous disciplines and < 0.01 for surgery and otorhinolaryngology). China contributed the greatest number of publications (n = 622, 21.3%), followed by South Korea (n = 478, 16.4%) and the United States (n = 468, 16.0%). @*Conclusion@#Radiology produced the most publications for thyroid US than any other discipline. Radiology authors published more notably on imaging diagnosis compared to other topics and in journals with lower impact factors compared to authors in other disciplines.

Perfil de la producción científica sobre anquiloglosia / Profile of scientific production on ankyloglossia

Este trabajo tiene como objetivodescribir el perfil de la producción científica referente a anquiloglosia. Para ello fuerealizada una investigación por medio de la búsqueda de artículos científicos indexados en las bases de datos electrónicas LILACS y PUBMED. Para la revisión bibliométrica fueron considerados y tabulados los datos referidos al año de publicación, tipo de estudio y nivel de evidencia. Los datos fueron discutidos sobre la óptica cuantitativa y de valores representativos. Los primeros resultados permitieron analizar 651 estudios publicados. La mayor parte de las investigaciones sobre anquiloglosia encontradas correspondieron a estudios de tipo descriptivo y serie de casos (49,31%) seguido de relato de caso, investigación in vitro, en animales y revisión de literatura (24,27%), cohorte y casos-controles (11,98%), opinión de experto (11,68%), ensayo clínico randomizado (1,54%) y revisión sistemática (1,22%). Pocos estudios abordaron complicaciones durante o después de la realización de la cirugía para liberación del frenillo lingual. En conclusión, la producción científica sobre anquiloglosia ha mostrado un creciente aumento en los últimos 28 años, siendo publicados estudios con nivel de evidencia 1, 2 y 3, cuyo mayor enfoque fue realizado en la cirugía para la liberación del frenillo lingual.

This work aims to describe the profileof scientific production referring to ankyloglossia. For this an investigation was carried out by searching for scientific articles indexed in the electronic databases LILACS and PUBMED. For the bibliometric review, the data referring to the year of publication, type of study and level of evidence were examined and tabulated. The data were discussed on the quantitative and representative values optics. The first results allowed to analyzic 651 published studies were analyzed. Most of the research on tongue tie found correspond to descriptive studies and case series (49.31%), followed by case reports, in vitro research, in animals and literature review (24.27%), cohort and cases and controls (11.98%), specialist opinion (11.68%), randomized clinical trials (1.54%) and systematic reviews (1.22%). Few studies addressed complications during or after lingual frenulum release surgery. In conclusion Scientific production on ankyloglossia has shown an increasing increase in the last 28 years, with studies with evidence levels 1, 2 and 3 being published, whose main focus was the performance of surgery to release the lingual frenulum.

Odontodisplasia Regional y Agenesia de Premolares. Reporte de un Caso / Regional Odontodysplasia and Premolar Agenesis. Case Report

RESUMEN: La odontodisplasia regional (OR) es una alteración en el desarrollo, no hereditario y que afecta tanto la dentición temporal como la dentición definitiva. Involucra a los tejidos mesodérmicos y ectodérmicos de los dientes lo que es condescendiente con hallazgos clínicos, radiográficos e histológicos. Su etiología aun es desconocida y se presenta mayoritariamente en mujeres. Clínicamente puede afectar al maxilar, a la mandíbula o ambas arcadas pero generalmente solo se ve comprometida una ellas, principalmente el más afectado es el hueso maxilar. Radiográficamente se observa una pobre diferencia entre los tejidos del esmalte y la dentina, siendo tejidos menos radiopacos que su contraparte sana generando un aspecto descrito como "diente fantasma". Histológicamente se observan zonas hipocalcificadas del esmalte con un orden de prismas irregulares mientras que la dentina se observa con un número reducido de túbulos dentinarios y de consistencia más fibrosa en su zona coronal. El tratamiento de la OR es controversial ya que su incidencia es baja y la literatura al respecto no es clara. El objetivo de este manuscrito, fue reportar un caso de OR y revisar la literatura relacionada. Presentamos un caso de OR en una paciente de 12 años que presenta ausencia de los dientes 2.4, 2.5 y 2.6; restos radiculares y agenesia de los dientes 3.5 y 4.5. Se describirán sus aspectos clínicos, radiográficos e histológicos. Se realizó una búsqueda sistemática en las siguientes bases de datos: Clínical key, Science Direct, PubMed y SciELO.

ABSTRACT: Regional odontodysplasia (RO) is a variation in the development; it is not hereditary and it affects both deciduous and permanent dentition. It involves the mesodermal and ectodermal tissues of dental pieces, and coincides with clinical, radiographic and histological findings. Its etiology is still unknown and it reportedly occurs mostly in women. Clinically it can affect the maxilla, mandible or both arches but generally only one is compromised, mainly the maxilla which is affected the most. Radiographically there is limited difference between enamel and dentin tissue, which is less radiopaque than their healthy counterpart, generating an aspect described as "phantom tooth". Histologically hypocalcified areas of the enamel are observed with an irregular order of prisms while the dentine is observed with a reduced number of dentinal tubules and more fibrous consistency in the coronal area. RO treatment is controversial since its incidence is low and the literature on these events is not clear. The aim of this manuscript was to report a case of RO and review related literature. We present a case of RO in a 12-year-old patient who presents absence of parts 2.4.2.5 and 2.6; radicular remains and agenesis of parts 3.5 and 4.5. Its clinical, radiographic and histological aspects are described. A systematic search was carried out in the following databases: Clinical key, Science Direct, PubMed and SciELO.

Research Areas and Trends in Articles on Pediatric and Adolescent Tuberculosis in Korea

PURPOSE: Despite the decline in tuberculosis (TB) incidence and mortality rates in the Republic of Korea through a national TB control program, TB remains one of the most critical infectious diseases in Korean children. We investigated the trends and research areas of published articles on TB in Korean children and adolescents. METHODS: In 6 Korean and overseas databases, we searched titles and abstracts including “tuberculo*” or “TB,” “child*” or “adolescen*” or “neonat*” or “infant*” or “pediatric*,” and “korea*.” The publication type, publication year, research areas, journal title, and research subjects were analyzed. RESULTS: Out of the 257 searched documents, 120 papers were included in the analysis. Of these, 82 were original articles (68.3%), 33 case reports (27.5%), 4 review articles (3.3%), and 1 guideline (0.8%). In the original articles, the most common subject of studies was the clinical characteristics of patients with TB (36.6%), followed by diagnostics (29.3%), contact investigations (9.8%), epidemiology (6.1%), treatment (4.9%), vaccine (3.6%), latent TB infection (3.6%), complications (3.6%), and surveys on perception of TB (2.4%). From 1962, 4 articles were published in the 1960s, 10 articles in the 1970s, 11 articles in the 1980s, 22 articles in the 1990s, 26 articles in the 2000s, and 47 articles since 2010. CONCLUSIONS: The amount of research on TB in Korean children has increased over the past 5 decades; however, it has mainly focused on the clinical characteristics and diagnostics. Research in different areas, such as treatment and vaccine, is needed in the future.

Caracterización bibliométrica de la producción científica de la Facultad de Estomatología Raúl González Sánchez, 2011-2015 / Bibliometric characterization of scientific production at Raúl González Sánchez dental school, 2011-2015

Introducción: el estudio bibliométrico es una herramienta que permite el análisis de la producción científica de las instituciones. Objetivo: caracterizar el comportamiento bibliométrico de la producción científica de la Facultad de Estomatología entre 2011-2015. Métodos: Se realizó un estudio bibliométrico descriptivo. El universo quedó conformado por 696 publicaciones realizadas en el periodo de estudio controladas en las bases de datos. Las variables estudiadas fueron: publicaciones por año, tipo de publicación, tipo de temáticas abordadas, lugar de publicación, categoría docente del primer autor y productividad por autores. Resultados: en el periodo se efectuaron 696 publicaciones científicas, el 44,2 por ciento correspondieron al tema clínico-patológico y el 18,5 por ciento al pedagógico. El 64,5 por ciento fueron artículos originales, el 17,5 por ciento revisiones bibliográficas y el 9,3 pr ciento presentaciones de casos, y las semblanzas y los editoriales con el 1,2 por ciento, respectivamente. El 48,4 por ciento de los primeros autores tenían categoría de profesor auxiliar. El 53,4 por ciento de los trabajos se publicó en revistas científicas. Diez profesores fueron los más productivos con un total de 101 artículos, para un 18,0 por ciento del total. Conclusiones: La producción científica se mantuvo en niveles similares por años. Más de la mitad de los artículos se publicó en revistas arbitradas; prevalecieron los artículos de temas clínico-patológico y pedagógico; la mayoría de los artículos fueron artículos originales; predominaron los primeros autores con la categoría de profesor auxiliar. Los profesores más productivos de la facultad tuvieron a su cargo casi la quinta parte de las publicaciones(AU)

Introduction: bibliometric studies are tools allowing analysis of the scientific production of institutions. Objective: characterize the bibliometric behavior of the scientific production at the School of Dentistry in the period 2011-2015. Methods: a descriptive bibliometric study was conducted. The study universe was composed of 696 papers published in the study period and included in the corresponding databases. The variables analyzed were publications per year, publication type, topic dealt with, place of publication, teaching category of the first author and productivity per author. Results: a total 696 scientific papers were published in the period, 44.2 percent of which dealt with clinical pathology and 18.5 percent with teaching. 64.5 percent were original papers, 17.5 percent were bibliographic reviews, 9.3 percent were case reports, 1.2 percent were bibliographic sketches and 1.2 percent were editorials. 53.4 percent of the first authors were Associate Professors. 52.4 percent of the papers had been published in scientific journals. Ten professors were the most productive with 101 papers, for 18.0 percent of the total number. Conclusions: scientific production has remained at a similar level for years. More than half the papers were published in peer-reviewed journals. There was a prevalence of clinical pathology and teaching subjects. Most of the papers were original. A predominance was found of first authors with the category of Assistant Professor. The most productive professors at the school published almost one fifth of all the papers(AU)

Efetividade dos princípios do sistema único de saúde na atenção primária à saúde: revisão sistemática / Effectiveness of the principles of the unified health system in primary health Care: systematic review of the literature

Este estudo teve como objetivo realizar uma revisão sistemática das publicações científicas que buscavam identificar a efetividade ou não dos princípios doutrinários e organizativos do Sistema Único de Saúde na Atenção Primária à Saúde e nos serviços por ela englobados no Brasil. Realizou-se uma revisão sistemática de literatura, com busca na Biblioteca Virtual em Saúde, baseada nos estudos publicados nos últimos dez anos (2006-2015), a partir dos descritores Atenção Primária à Saúde, Direito à Saúde, Efetividade e Sistema Único de Saúde, resultando em 29 publicações relacionadas ao tema. Estas foram analisadas por ano de publicação, tipos de estudo, princípio estudado, tipos de serviço, usuários e/ou categorias profissionais analisadas e regiões e Unidade Federadas analisadas. Obteve-se que os princípios doutrinários não foram plenamente efetivados pela presença de conduta profissional centralizada no modelo biomédico, houve descompasso entre as necessidades do usuário e o que era oferecido e falta de recursos humanos e de acesso aos serviços pelos usuários. Em relação aos princípios organizativos, foi exposto que a precária acessibilidade ao atendimento especializado, a falta de apoio do Estado no processo de municipalização, a baixa cobertura de serviços no espaço geográfico e um processo democrático deficitário prejudicaram sua efetividade. Os resultados dos estudos identificam a importância dos princípios e de sua efetividade para a qualificação dos serviços e satisfação dos usuários e profissionais neles inseridos, o que não foi plenamente encontrado nos serviços da Atenção Primária à Saúde.

This study aimed to carry out a systematic review of the scientific publications that sought to identify the effectiveness, or not, of the doctrinal and organizational principles of the Unified Health System in Primary Health Care and in the services it encompasses in Brazil. We conducted a systematic review of the literature, searching in the Virtual Health Library, based on studies published in the last ten years (2006-2015), using the descriptors Primary Health Care, Right to Health, Effectiveness, and Unified Health System, resulting in 29 publications related to the topic. They were analyzed by year of publication, type of study, principle studied, types of service, users and/or professional categories analyzed, and Regions and Federated Unit (State) analyzed. It was found that the doctrinal principles were not fully realized by the presence of professional conduct centralized in the biomedical model. There was a mismatch between the needs of the user and what was offered, and a lack of human resources and access to services by the users. Regarding the organizational principles, it was shown that the precarious accessibility to specialized services, the lack of government support in the municipalization process, the low coverage of services in the geographic space, and an unsatisfactory democratic process jeopardized its effectiveness. The results of the studies identified the importance of the principles and their effectiveness for the qualification of the services and satisfaction of users and professionals included in it, which was not fully found in Primary Health Care services.

Pneumonia caused by Bordetella bronchiseptica in two HIV-positive patients / Pneumonia causada por Bordetella bronchiseptica em dois pacientes HIV-positivos

ABSTRACT: CONTEXT AND OBJECTIVE: Bordetella bronchiseptica (BB) is a Gram-negative coccobacillus responsible for respiratory diseases in dogs, cats and rabbits. Reports on its development in humans are rare. However, in immunosuppressed patients, especially in those with the immunodeficiency virus (HIV), BB can cause severe pulmonary infections. We report on two cases of pneumonia caused by BB in HIV-positive male patients in a university hospital. CASE REPORT: The first case comprised a 43-year-old patient who was admitted presenting chronic leg pain and coughing, with suspected pneumonia. BB was isolated from sputum culture and was successfully treated with trimethoprim/sulfamethoxazole in association with levofloxacin. The second case comprised a 49-year-old patient who was admitted presenting fever, nausea, sweating and a dry cough, also with suspected pneumonia. BB was isolated from sputum culture, tracheal secretions and bronchoalveolar lavage. The disease was treated with ciprofloxacin but the patient died. CONCLUSION: BB should be included in the etiology of pneumonia in immunodeficient HIV patients. As far as we know, these two were the first cases of pneumonia due to BB to occur in this university hospital.

RESUMO CONTEXTO E OBJETIVO: Bordetella bronchiseptica (BB) é um cocobacilo Gram-negativo responsável por causar doenças no trato respiratório de cães, gatos e coelhos. São raros os relatos do desenvolvimento desse microrganismo em seres humanos. Porém, em pacientes imunodeprimidos, especialmente nos portadores do vírus da imunodeficiência humana (HIV), a BB pode causar infecções pulmonares graves. Nós relatamos dois casos de pneumonia por BB em pacientes do sexo masculino, HIV-positivos em um hospital universitário. RELATO DE CASO: No primeiro caso, o paciente de 43 anos foi internado apresentando dor crônica nos membros inferiores e tosse com suspeita de pneumonia. Na cultura de escarro, foi isolado BB, e a infecção foi tratada com sucesso com a associação de sulfametoxazol/trimetroprima e levofloxacino. No segundo caso, o paciente de 49 anos foi internado apresentando febre, náuseas, sudorese e tosse seca, também com suspeita de pneumonia. Das culturas de escarro, secreção traqueal e lavado bronco-alveolar, foi isolado BB, infecção tratada com ciprofloxacino: porém, o paciente foi a óbito. CONCLUSÃO: BB deve ser incluído na etiologia de pneumonia em pacientes imunocomprometidos com HIV. Pelo que é de nosso conhecimento, estes dois relatos foram os primeiros casos de pneumonia por BB que ocorreram neste hospital universitário.

Progress on Post Traumatic Epilepsy and Its Forensic Evaluation / 法医学杂志

Post traumatic epilepsy （PTE） refers to the epileptic seizures after traumatic brain injury. Organic damage can be found by imaging examination, and abnormal electroencephalogram can be detected via electroencephalogram examination which has the similar location of the brain injury. PTE has the characteristics of low incidence, absence of case reports, and easy to exaggerate the state of illness, which add difficulties to the forensic identification. This paper reviews the status of epidemiology, pathogenesis, clinical treatment and forensic identification for PTE.

Progress on Post Traumatic Epilepsy and Its Forensic Evaluation / 法医学杂志

Post traumatic epilepsy(PTE)refers to the epileptic seizures after traumatic brain injury. Or-ganic damage can be found by imaging examination, and abnormal electroencephalogram can be detected via electroencephalogram examination which has the similar location of the brain injury. PTE has the characteristics of low incidence, absence of case reports, and easy to exaggerate the state of illness, which add difficulties to the forensic identification. This paper reviews the status of epidemiology, patho-genesis, clinical treatment and forensic identification for PTE.

Malignant ventricular tachycardia in acromegaly: a case report / Taquicardia ventricular maligna na acromegalia: relato de caso

CONTEXT: In patients with acromegaly, cardiovascular complications are the main cause of death; sudden death has been associated with ventricular tachyarrhythmias. In other patients with life-threatening malignant ventricular tachyarrhythmias, surgical placement of an implantable cardioverter-defibrillator (ICD) has proved highly effective in reducing sudden death rates. CASE REPORT: The present article reports the case of a 50-year-old male acromegalic patient who presented symptoms of syncope induced by ventricular tachycardia. An ICD was surgically implanted and a pituitary adenoma, which was responsible for the acromegaly, was completely removed in the same procedure. The surgery was successful and the ventricular arrhythmias were effectively terminated. During six months of follow-up, no documented arrhythmic episodes occurred. CONCLUSION: In patients with acromegaly, malignant ventricular tachyarrhythmia might be effectively controlled by implantation of an ICD and surgical removal of the pituitary adenoma. .

CONTEXTO: As complicações cardiovasculares são a principal causa de morte em pacientes com acromegalia, e a morte súbita tem sido associada a taquiarritmias ventriculares. Em outros pacientes com risco de vida por taquiarritmias ventriculares malignas, a aplicação cirúrgica de um cardioversor-desfibrilador implantável (CDI) provou ser altamente eficaz na redução das taxas de morte súbita. RELATO DE CASO: O presente artigo relata o caso de um paciente acromegálico de 50 anos de idade e do sexo masculino, que apresentava sintomas de síncope induzida por taquicardia ventricular. Foi implantado cirurgicamente nesse paciente um CDI e na mesma intervenção cirúrgica foi completamente removido um adenoma hipofisário responsável pela acromegalia. A cirurgia foi bem-sucedida e o paciente deixou de sofrer de arritmias ventriculares. Durante seis meses de acompanhamento, não se documentaram, nesse paciente, episódios arrítmicos. CONCLUSÃO: A taquiarritmia ventricular maligna pode ser efetivamente controlada em pacientes com acromegalia pela implantação de um CDI combinado com a remoção cirúrgica do adenoma hipofisário. .

Clinicopathologic analysis of two cases of mucoepidermoid carcinoma of the skin / 中华皮肤科杂志

Objective To describe the clinicopathologic features of two cases of mucoepidermoid carcinoma of the skin.Methods Two cases of mucoepidermoid carcinoma of the skin were analyzed histopathologically using hematoxylin and eosin (HE) staining,alcian blue-periodic acid Schiff (AB-PAS) staining and immunohistochemical staining.Relavant literature was reviewed.Results Histopathological examination showed that the tumor was subcutaneously located in both cases,with epidermoid cells and intermediate cells arranged in sheets or nests,as well as different sizes of glandular structures lined by mucinous columnar epithelium in some areas.Both tumors had a relatively clear boundary with peripheral invasive growth and no obvious capsules.Immunohistochemical examination showed positive staining for carcinoembryonic antigen (CEA),high and low molecular weight cytokeratin (CK(H) and CK(L)).The cytoplasm of mucous cells was stained blue with,and mucus was visualized after,AB-PAS staining.Conclusions Primary mucoepidermoid carcinoma of the skin is a kind of malignant tumor arising from skin appendages,whose diagnosis depends on histological and immunohistochemical examination.

POEMS syndrome associated with multicentre Castleman's disease and thrombocytosis: a case report / 中华皮肤科杂志

A rare case of POEMS(polyneuropathy,organomegaly,endocrinopathy,M-band,skin changes) syndrome associated with multicentre Castleman's disease and thrombocytosis is reported in a 48-year-old man.Three years prior to the presentation,he developed a series of symptoms without obvious triggers,including persistent myalgia and myasthenia of extremities,abnormal skin sensation (burning),gradually increased roughness and pigmentation of skin,thickening and mild induration of lower-extremity skin giving an ichthyosiform appearance.Two years prior to the presentation,electromyography indicated peripheral neuropathy,and computed tomography(CT) revealed hepatomegaly,splenomegaly,lymphadenectasis,ascites,hydrotborax and hydropericardium.Multiple biopsies of lymph nodes indicated an obvious vascular proliferation between lymphoid follicles with the involvement of lymphoid follicles,which,together with the histochemical findings and special staining results,was suggestive of hyaline-vascular type Castleman's disease.Three months prior to the presentation,painful erythema emerged and gradually spread in both thighs,with a progressive deepening in the colour of central lesions,and flushing of periphery lesions.Two months prior to the presentation,a soybean-sized nodule arised in the erythema on the right inner thigh,which was then ulcerated with a little pus left and a shallow ulcer formed.Physical examination revealed a mild atrophy of lower-extremity muscles,with muscle strength graded as 5 for bilateral upper extremities and as 4 for bilateral lower extremities.The skin was rough,diffusely pigmented and thickened,giving an ichthyosiform appearance,which was more apparent in the extremities than in the trunk.An atropurpureus patch sized 5 cm × 2 cm was seen respectively in bilateral inner thighs; the surface of the central lesions was coarse and covered with dry crusts and scales,giving a rough paperlike appearance,and the peripheral lesions were surrounded by a faint prunosus halo,which faded on pressure.Laboratory examination found hypothyroidation,reduced insulin secretion,abnormal calcium and phosphorus metabolism,and progressive thrombocytosis.Immunofixation electrophoresis showed that the serum was weakly positive for IgG type M protein.This patient was diagnosed with POEMS syndrome associated with multicentre Castleman's disease and thrombocytosis.After treatment with methylprednisolone and thalidomide,the main symptoms of POEMS syndrome were improved,whereas thrombocytosis progressively aggravated.

Trichogerminoma: a case report / 中华皮肤科杂志

A case of benign follicular neoplasm-trichogerminoma-is reported.A 48-year-old man presented with a 10-year history of asymptomatic subcutaneous nodule in the chest.Histological examination revealed a well-circumscribed lesion composed of variously sized lobuli and cysts in the deep dermis and separated from the surrounding tissue by a fibrous capsule.Most lobuli consisted of concentrically arranged clear cells in the central area and basophilic cells in a palisade arrangement in the peripheral area.The tumor cells displayed a multi-directional differentiation toward hair bulb,inner root sheath,outer root sheath and infundibulum of hair follicles.Immunohistochemically,the tumor cells expressed AE1/AE3,CK5/6 and CK17,but were negative for CK20 or CK7.There was a sharp contrast in immunohistochemical findings between the central clear cells and peripheral basophilic cells.Based on the histological and immunohistochemical features,a diagnosis of trichogerminoma was made.

Malignant atrophic papulosis with neurological involvement / 中华皮肤科杂志

A 48-year-old female presented with a 6-year history of papules and plaques all over the body and with 1-year history of blurred vision in the right eye.Physical examination showed porcelain-white atrophic papules with peripheral erythematous halo and telangiectnsia.She also suffered from exotropia,visual deterioration,visual field defects of the right eye,as well as numbness of the left index finger,thumb and right anterior tibia.Skin biopsies of abdominal lesions revealed dermal necrosis with mucoid degeneration,inflammatory infiltration predominated by lymphocytes around several small blood vessels and occlusion of some blood vessels in deep dermis.Colonoscopy of the whole colon demonstrated scattered patches of hyperemia and erosions with the formation of shallow ulcers.Nerve electromyologram revealed damage to the nerves of right quadriceps femoris muscles.Fecal analysis showed that occult blood was strongly positive.A diagnosis of malignant atrophic papulosis was made based on the characteristic clinical presentation,laboratory and histopathological findings.She was treated with dipyridamole and aspirin for three months,which resulted in no clinical improvement or deterioration.

Mycosis fungoides palmaris et plantaris: a case report / 中华皮肤科杂志

A 41-year-old man presented with recurrent pustules and scales on both hands and feet for 1year and with neoplasm on the left toe for 4 months.On physical examination,there were multiple irregular rufous patches with lamellar white scales on bilateral thumbs,forefingers,thenar eminence,toes,and the onethird anterior part of bilateral soles.Deep-seated pustules and vesicles arising on some erythematous patches were disrupted with the formation of punctiform or patches of erosions covered by yellow brown crusts.Some toenails and fingernails were thickened and deformed.An obviously tender neoplasm measuring 4 cm × 3.5 cm× 3.5 cm was observed in the anterior part of the left fourth toe,which was a little indurated and obviously tender with superficial erosion,large amount of purulent exudates and fresh granulation tissue mixing to form black and thick blood crusts on the surface.Biopsy of the nail bed of the right index finger and neoplasm on the left toe revealed a diffuse,dense,and mixed infiltrate of small- to medium-sized atypical lymphocytes and a few inflammatory cells.Meanwhile,epidermotropism and Pautrier's microabscess were visible.Immunohistochemistry showed that the abnormal lymphocytes were positive for leukocyte common antigen (LCA),Vimentin,CD3,CD45RO,CD4,Bcl-2,partly positive for CD8,CD5,CD10,Ki-67 (＞ 80％ ),but negative for CD79a,CD20,CD30,cytokeratin,S-100,Bc1-6,anaplastic lymphoma kinase (ALK),HMBMS,CD1a or P63.Based on the above findings,a diagnosis of mycosis fungoides palmaris et plantaris was made.

Ulerythema ophryogenes: a case report / 中华皮肤科杂志

A case of ulerythema ophryogenes (UO) is reported.A 12-year-old boy presented with erythema and follicular papules on the eyebrows and cheeks for 7 years.The lesions started as follicular papules surrounded by erythema,then spread symmetrically to the cheeks and forehead followed by the loss of eyebrows.There was no complaint of pruritus.Physical examination showed pinhead- to grain-sized,smooth,slightly indurated follicular hyperkeratotic papules surrounded by erythematous halo on the eyebrows,forehead and cheeks.Both eyebrows were nearly completely lost.Histological analysis of lesions from eyebrows revealed dilated follicular infundibulum with orthokeratotic plugs,sparse perivascular and perifollicular lymphohistiocytic infiltrate,widened and sclerotic collagen fibers in the dermis.According to the clinical manifestations and histopathological findings,the patient was diagnosed with ulerythema ophryogenes,and given oral vitamin A 2.5 million unit once a day,vitamin E 100 mg once a day,topical vitamine E cream twice a day,0.025％tretinoin ointment once at night.Two weeks later,the lesions improved.

Cutaneous intravascular NK/T cell lymphoma: a case report / 中华皮肤科杂志

An 18-year-old female presented with painful erythema and nodules on both legs for more than 2 years.Dermatological examination showed irregularly sized,mildly indurated,tender,deep subcutaneous nodules arising in diffused infiltrated dark erythematous patches in the inner and posterior region of the left leg.Histopathology showed no significant changes in the epidermis.There were perivascular lymphoid cell infiltrates in the dermis and subcutis.Multiple sites of necrosis of blood vessel walls with vascular occlusions were noted.The lumens of some blood vessels were filled with lymphocytes,among which were many atypical cells with hyperchromatic nuclei and pathologic mitotic figures.Immunohistochemistry showed that lymphocytes in the cavities of blood vessels were positive for CD3(+++),CD3ε(+++),CD2(+),CD56(+++),granzyme B(+++),perforin(+++),CD30(+),Ki67 (+++,100％ ),but negative for CD20,CD5,CD7,CD4,CD8,TdT,anaplastic lymphoma kinase,early membrane antigen (EMA) or pan cytokeratin (pCK).The endothelial cells lining the blood vessels stained positively for CD34.The intravascular lymphocytes were also positive for EBER1/2 by in situ hybridization.A diagnosis of cutaneous intravascular NK/T cell lymphoma was made.

Skin adnexal tumor with multipote ntial differentiation complicated by nevus spilus: a case report / 中华皮肤科杂志

A 41-year-old female patient developed round,bright yellow patches on the left calvarial region without obvious precipitating factors 40 years prior to the presentation,which gradually grew to form plaques with age.Two years prior to the presentation,nipple-like lesions appeared in the calvarial and temporal region with an erythematous and wet surface; concurrently,black masses developed in the left temporal region and gradually enlarged with central ulceration but no subjective symptoms.At about 1 year of age,pitchy macules developed on the light tan patches located on the left jaw,posterior and anterior neck,trunk and upper limbs,and gradually increased in quantity and size with the involvement of the homolateral dorsal hand and gradual appearance of papules.Skin examination revealed two well-marginated,indurated,bright red neoplasms sized 3 cm × 2 cm and 2 cm × 1 cm respectively,with erosive and cauliflower-like surface; black or pink papules were scattered between these neoplasms.There was a ring-shaped black mass sized 1.5 cm × 1.5 cm in the left temporal region with central ulceration.Pitchy tough macules and papules were observed on the light tan patches located in the left cheek,lower mandible,posterior and anterior neck,protothorax,shoulder and back,upper limbs and dorsal hand.Based on the histopathology of multiple lesions,the cauliflower-like lesions on the head were diagnosed as syringocystadenoma papilliferum,the yellow plaques as syringocystadenoma papilliferum complicated by sebaceous adenoma,the black proliferative lesions in the temporal region as trichoblastoma accompanied by basal cell epithelioma,the black papuloid lesions and brown maculopapuloid lesions on the lower mandible as nevus spilus.The patient was diagnosed with skin adnexal tumor with multipotential differentiation (syringocystadenoma papilliferum,sebaceous adenoma,trichoblastoma and basal cell epithelioma)accompanied by nevus spilus.

Intravascular NK/T-cell lymphoma firstly manifesting as gastrointestinal tract symptom and complicated by skin lesions: a case report / 中华皮肤科杂志

A 51-year-old man presented with a 4-month history of upper abdominal distending pain and 1-month history of cutaneous nodules and plaques on the neck, trunk and bilateral thighs. The patient underwent many gastrointestinal tract examinations in several local hospitals, and symptomatic treatment did not work. The biopsy of nodules on the abdomen revealed medium- to large-sized atypical lymphoid cells within numerous small vessels in lower dermis and subcutaneous fat tissue. Additionally, the atypical cells were present exclusively within vascular lumina. Immunohistochemical labeling showed the reactivity of neoplastic cells to CD2, CD99, CD3ε, CD43, CD56, Epstein-Barr virus-encoded small nuclear RNAs (EBER), and cytotoxic proteins such as T-cell intracellular antigen-1 (TIA-1) and perforin, but not to CD4, CD8, CD20, CD79a,CD30, cytokeratin (CK), S100, or CD68. The endothelial cells lining the involved vessels exhibited the reactivity to CD31 and CD34. Based on the above findings, the patient was diagnosed with intravascular NK/T-cell lymphoma firstly manifesting as gastrointestinal tract symptom and complicated by skin lesions. Following combined chemotherapy with cyclophosphamide, daunorubicin, vincristine, prednisone and etoposide, the patient experienced a quick and satisfactory recovery and the follow-up still continued.