Multiple pilomatricomas in twins with Rubinstein-Taybi syndrome

Abstract Pilomatricomas are benign tumors originating from the capillary matrix, which may present as solitary lesions or, less commonly, multiple. Myotonic dystrophy and familial adenomatous polyposis are the most frequently associated disorders with multiple pilomatricomas. There are few reports relating these tumors to other genetic syndromes. Rubinstein-Taybi syndrome is a rare autosomal dominant disorder characterized by intellectual disability and typical dysmorphic characteristics. There are five case reports relating to multiple pilomatricoma to Rubinstein-Taybi syndrome, an association that needs to be clarified. For this reason, we report the first case of multiple pilomatricoma in monozygotic twins with typical Rubinstein-Taybi syndrome.

A case of small cell neuroendocrine carcinoma of ovary / 中国综合临床

Objective@#Small cell neuroendocrine carcinoma of the ovary is a kind of ovarian cancer with a very low incidence.Its clinical manifestations are not obvious.The diagnosis should be based on the pathology and neuroendocrine indicators, and its primary nature should be determined.The main treatment is operation combined with platinum based chemotherapy.The survival period is related to clinical stage and treatment plan.The patient was hospitalized for 2 days because of the aggravation of abdominal distention and pain for half a year.The diagnosis of adnexal mass was confirmed by pathology.After three cycles of neoadjuvant chemotherapy (etoposide+ cisplatin), the patients underwent abdominal " total hysterectomy+ greater omentum resection+ appendectomy+ right pelvic wall peritoneal biopsy+ mesenteric biopsy" . After the operation, the patients received three cycles of EP chemotherapy, and they have been followed up for 15 months.

A brief analysis of projects completed at the end of 2017 and funded by National Natural Science Foundation of China in the field of skin and its appendages / 中华皮肤科杂志

Objective To analyze projects completed at the end of 2017 and funded by National Natural Science Foundation of China (NSFC) in the field of skin and its appendages,in order to provide references for preparing concluding reports and making discipline development strategies in the future.Methods The concluding reports of projects completed at the end of 2017 and funded by National Natural Science Foundation of China in the field of skin and its appendages were collected from the Internet-based Science Information System.The achievements were analyzed,including journal articles,patents,talent training and monographs.Results A total of 91 projects were completed at the end of 2017 in the field of skin and its appendages,and the total cost of funds was 43.51 million RMB Yuan.A total of 452 journal articles were published,including 324 articles in the Science Citation Index (SCI)-indexed journals and 42 in the A Guide to the Core Journal of China-indexed journals.Additionally,0.74 SCI journal article and 0.10 Chinese core journal article could be produced per 100 000 RMB Yuan.The average impact factor of journals publishing these SCI articles was 3.972,and in average,100 000 RMB Yuan yielded 1 article in the journal with an impact factor of 2.92.Of the 324 published articles,46 and 102 were published in journals ranked within the first (Q1) and second (Q2) quartile,respectively,by Chinese Academy of Sciences.In all the SCI articles,14 were published in journals with impact factor of ＞ 10.There were 4 institutions with more than 5 completed projects,and a total of 129 (39.84％) SCI articles were published.Published SCI articles in the field of immune skin diseases accounted for the highest proportion (148/324,45.68％).A total of 152 talents were trained.Conclusions The support of NSFC in basic researches in the field of skin and its appendages has led to substantial achievements.More attention should be paid to the writing of concluding reports.Innovation consciousness should be raised,and project leaders should make more original and leading achievements in the future.

Gender Determination Using Peripheral Blood Smear

Background: Sex chromatin is a chromatin mass of 1 micron size usually seen at the periphery of nucleus infemales. The term sex chromatin comprises of two superficially dissimilar structures known as Barr body inepithelial cells, other tissue cells and as Drumstick appearance in polymorphonuclear leucocytes. Aim of thestudy is gender determination by drumstick appearance of neutrophils using Leishman’s stain and Field stain.Materials and Methods: The present study included 60 individuals ranging from 20-50 age group of both thesexes to observe the morphology of sex chromatin in neutrophil by using Leishman’s and Field stain for thegender determination and comparison between the 2 staining procedure, using research microscope.Results: Presence of drumsticks, sessile nodule and non specific appendages like racket, tag, hook, minor lobe,small club, were found in neutrophils. In males 37% of drumstick, 7% of sessile nodule, 20% of racket structure,3% small club, 13% minor lobe, 7% hook, 3% tag, was seen and in females 10% of drumstick, 60% of sessilenodule, 17% of racket structure, 7% small club, 1% minor lobe, 0% hook, 3% tag, was seen and field’s stainshowed better results in appreciating the morphology of neutrophil.Conclusion: Observation on morphology and comparison between 2 different stain presented a moderate agreementin gender determination.

Multiple vulvar dilated pores in a postmenopausal woman

Abstract: Winer's dilated pore is an infrequent appendageal tumor characterized by a giant comedone on the face, neck, and upper trunk in adults. We report a 57-year-old woman who developed multiple asymptomatic black papules on both labia majora. Histopathology showed grouped dilated follicles lined by keratinizing squamous epithelium in the superficial dermis. The superficial lining epithelium and interfollicular epidermis were atrophic, while the deep epithelium showed mild proliferation and melanin pigmentation with a few short projections extending into the surrounding dermis. We diagnosed multiple Winer's dilated pores based on late-onset lesions and pathological features. This patient may represent the first case of multiple vulvar Winer's dilated pores. We suggest that electrocautery may be effective for treating this type of superficial entity.

Dermoid cyst in the floor of the mouth

@#Dermoid cysts are rare developmental benign lesions that arise as a result of ectodermal differentiation of multipotent cells. Histologically the cysts are classified as dermoid, epidermoid and teratoid cysts depending on the presence of adnexal structures and derivatives of germ layers. When dermoid cysts appear in the floor of the mouth they can cause difficulty in deglutition and respiration. The differential diagnosis of dermoid cyst could be infection, ranulaor tumor. Intraoral or extra oral approach for enucleation is the method of treatment. Dermoid cysts have a good prognosis with low malignancy and recurrence rate. A case report of a dermoid cyst in the floor of the mouth is presented in this paper along with clinical, histopathological evaluation and the treatment.

Carcinoma triquilemal, um tumor incomum: atualização sobre manejo e prognóstico / Trichilemmal carcinoma, an uncommon tumor: update on its management and prognosis

INTRODUÇÃO: O carcinoma triquilemal (CT) é uma neoplasia rara dos anexos cutâneos. Foi descrito pela primeira vez em 1968, como tricoleptocarcinoma, e tem incidência de 0,05% em pacientes submetidos a exame histopatológico após excisão de lesões cutâneas. Parece ser um tumor de baixa agressividade, porém, relatos na literatura colocam em dúvida tal comportamento indolente. OBJETIVOS: Oferecer uma atualização sobre manejo e prognóstico do CT. MÉTODOS: Pesquisa no PubMed e SciELO com os termos MeSH "trichilemmal carcinoma", "tricholemmal carcinoma", "adnexal skin tumor", "carcinoma triquilemal". RESULTADOS: Devido a sua raridade, a maior parte da literatura se baseia em relatos de caso, os quais na sua maioria corroboram a natureza indolente da doença. Excisão cirúrgica é o tratamento preconizado, com baixo risco de recidiva e baixa morbimortalidade. CONCLUSÃO: O comportamento da lesão e as condutas no tratamento do CT baseiam-se em casos isolados ou de pequenas séries de casos. Devido a sua baixa prevalência, uma colaboração multicêntrica agrupando um maior número de casos pode ajudar a definir melhor recomendações de tratamento, fisiopatologia e prognóstico. Excisão cirúrgica continua a ser o padrão-ouro de tratamento, com baixo risco de recidiva.

INTRODUCTION: Trichilemmal carcinoma (TC) is a rare neoplasm of skin appendages. It was first described in 1968 as tricoleptocarcinoma, and has an incidence of 0.05% in patients subjected to histopathological examination after excision of cutaneous lesions. TC has an indolent clinical course ; however, reports in the literature put in doubt this indolent behavior. OBJECTIVES: To provide an update on the management and prognosis of TC. METHODS:A search of the PubMed and SciELO databases by using with the MeSH terms "trichilemmal carcinoma", "tricholemmal carcinoma", "adnexal skin tumor", and "carcinoma triquilemal" was performed. RESULTS: Owing to the rarity of TC, most studies were case reports, which essentially corroborate the indolent nature of the disease. Surgical excision is the recommended treatment, and is associated with a low risk of recurrence and low morbidity and mortality. CONCLUSION: The behavior of the lesion and the procedure of treatment of TC are based on isolated cases or in a small series of cases. Because of its low prevalence, a multicenter collaboration of a greater number of cases can help define the best treatment recommendations, pathophysiology, and prognosis. Surgical excision remains the gold standard of treatment, and is associated with a low risk of recurrence.

Sexual Dimorphism in Morphology and Morphometry of Neutrophil Drumsticks.

Introduction: Sex chromatin is a chromatin mass of 1 micron size usually seen at the periphery of nucleus in females. In the literature majority reported its absence inmales while few reported its low incidence inmales. The term ‘sex chromatin’ comprises two superficially dissimilar structures the “Barr body” present in epithelial and other tissue cells and the “Drumstick” of the polymorphonuclear leucocytes. Materials and methods: The present study was conducted to observe the morphology, morphometry and percentage incidence of Drumsticks in the blood neutrophils of 110 individuals ranging from 17-30 age group and both sexes using a calibrated ocular/eye piece micrometer. Results: The percentage incidence of drumsticks including non-specific appendages as well as the total number of true drumsticks in females exceeds that in males. Four different types of nonspecific appendages-sessile nodules, racket structures, minor lobes and small clubs were found in the blood neutrophils along with the drumsticks. A higher percentage of non-specific appendages i.e.minor lobes (46.2%), racket structures (42.3%), and small clubs (11.5%) were observed in males and sessile nodules were found only in females. Conclusion: Observations on morphology, morphometry and percentage incidence of polymorphonuclear drumsticks presented a valuable data on sex differences.

Two-dimensional ultrasound imaging of atrial appendages in normal fetuses / 中华超声影像学杂志

Objective To understand demonstration methods and ultrasonographic features of left and right atrial appendages in normal fetuses.Methods 200 consecutive normal fetuses during gestation age 19-28 weeks from November 2011 through April 2012 entered the study.Ultrasonographic features and the demonstrated rates of atrial appendages at different incidence angle of ultrasonography and different gestational weeks were recorded and calculated,respectively.Results Atrial appendages of normal fetuses can be demonstrated on atrial appendages plane,which was little lower the view of parasternal great artery short axis.The display rate of left,right and left-right atrial appendages was 92％,68％,65.5％,respectively.When ultrasound beam entranced into chest from the right side,the display rate of left-right atrial appendages was highest(94.3％).The best time to observe atrial appendages was 22 to 24 week of pregnancy.The majority of left atrial appendages were fingerlike hook shapes,with narrower bases and longer bodys,and the endocardial surfaces were relatively smooth.Sometimes with incisures on external edges.Whereas the majority of right atrial appendages were obtuse-angled triangle shapes,with broad bases and shallower bodys.On endocardial surface the pectinate muscles were often seen,which looked like serrated echoes,sometimes prominent taenia sagittalis were noted in right atrial appendages.Conclusions Atrial appendages of normal fetuses can be demonstrated using two-dimensional ultrasonography on atrial appendages plane.The demonstrated rates of atrial appendages were different according to different incidence angle of ultrasonography and gestational weeks.There were some differences in ultrasonographic features between left and right atrial appendages,which is very helpful in determinating atrial situs.

Gene expression of the skin and sweat glands in different development stages of embryo / 中国组织工程研究

10.3969/j.issn.2095-4344.2013.24.008

Desarrollo de la Piel y sus Anexos en Vertebrados / Skin and Appendages Development in Vertebrates

La Piel y sus estructuras asociadas permiten a los seres vivos subsistir en los diferentes ambientes ecológicos. El desarrollo de la piel y sus anexos en diferentes especies repite patrones comunes. De suma importancia es la interacción epitelio-mesénquima como regulador inicial de este desarrollo. El evento crucial en la formación de anexos, es la aparición de una placoda ectodérmica, a la cual se le asocia una condensación de células dérmicas, expresándose proteínas como Sonic Hedgehog (SHH) y la proteína morfogenética del hueso (BMP) para luego dar forma al anexo de cada especie. En esta revisión describiremos las etapas sucesivas que transcurren en la formación de la dermis, epidermis y anexos, con énfasis en las proteínas que dirigen el proceso.

Skin and associated structures allow animals to survive in different ecological environments. The development of skin and appendages in different species has common patterns repeated. Of utmost importance is the epithelial-mesenchymal interaction as the initial controller development. The crucial event in the formation of appendages is the appearance of an ectodermal placode, which is associated with a condensation of dermal cells, expressing BMP and Sonic Hedgehog proteins and then give the way to each species appendages. In this review we describe the successive stages that take place in the formation of the dermis, epidermis and appendages, with emphasis on proteins that direct the process.

A Case of Peritoneal Loose Body in a Child / 대한소아소화기영양학회지

A peritoneal loose body is reported to develop because of torsion and separation of the epiploic appendages. The condition is usually symptomless and may be incidentally during abdominal surgery or autopsy. It usually occurs in middle-aged and elderly adults and is very rare in children. In this paper, we report a case of a peritoneal loose body in the pelvic cavity of a 10-year old-girl who presented with urinary frequency and left lower abdominal discomfort. A second plain X-ray film of the abdomen, obtained before surgery, in a different view than the first, revealed that the calcified mass had migrated to a lower position. The mass was laparoscopically resected, and histological examination revealed it to be a fibrotic nodule with central liquefaction and calcification.

A scanning electron microscopic study on the appendage morphology of Astacus leptodactylus (Eschscholtz, 1823) and Pacifastacus leniusculus (Daña, 1852) (Crustácea:Decapoda: Astacoidea)

This study compares the morphology of rostrum, pereipods 1,2,4 and mouthparts of juvenile Astacus leptodactylus with those oí Pacifastacus leniusculus. Differences in morphology were observed, in particular with regard to the mouthparts e.g. including setal armature and number of teeth on the mandible. In general, the shape of the rostra in the two species is similar in that both taper to a point with a pair of sharp spines distally. Laterally the rostrum of A. leptodactylus is bordered by a regular row of setae, which is not so well defined in P leniusculus. The observations also showed that in addition to an increase in size, changes in morphology in the feeding apparatus between the developmental stages of the two species were present. It was concluded that both species have similar rostra, but different setal patterns and there are differences between the two species in the armature of mouthparts as development progresses. Therefore, important differences in the morphology of mouthparts between P. leniusculus and A. leptodactylus and in the different stages of the species might cause a difference in the feeding behavior and food choice of the species.

Este estudio compara la morfología del rostro, pereiópodos 1,2,4 y piezas bucales de los Astacus leptodactylus jóvenes con los de Pacifastacus leniusculus. Se observaron las diferencias en la morfología, en particular, con respecto a las piezas bucales, por ejemplo incluyendo la armadura setal y el número de dientes en la mandíbula. En general, la forma del rostro en las dos especies es similar, tanto cónicas, como en punta, con un par de espinas distalmente. Lateralmente al rostro, A. leptodactylus está bordeada por un fila de setas, que no está tan bien definida en P leniusculus. Las observaciones también muestran que, además de un aumento en el tamaño, estaban presentes cambios en la morfología en el aparato masticatorio, entre las etapas de desarrollo de las dos especies. Se llegó a la conclusión que ambas especies tienen rostros similares, pero diferentes patrones setales y hay diferencias entre las dos especies en la armadura de piezas bucales como evolución del desarrollo. Por lo tanto, importantes diferencias en la morfología de piezas bucales entre P leniusculus y A. leptodactylus y en las distintas etapas de la especie podrían causar una diferencia en la conducta de alimentación y opciones de alimentación de la especie.

Influence of dermal analog composed with MSCs upon the differentiation potency of ES cell-derived epidermal stem cells / 基础医学与临床

Objective To explore the differentiation potency of ES cell-derived epidermal stem cells compounded by dermal analogs reconstructed with bone MSCs in hypodermis.Methods The dermal analogs were reconstructed with rat bone MSCs and compound gel-gelatin sponge.E14-ES cells,labeled with Hoechst 33342,were cocultured with human amnion.Four days later,epidermal stem cell clones were formed.The skin analogs,reconstructed with ES cell-derived epidermal stem cells and dermal analogs,were transplanted into 129 mice hypodermis.The differentiation tissue of skin analogs was sampled at 2,4,6,8 weeks.The sections were observed with HE staining,immunohistochemical and di-labeled immunofluorescence methods to test the expression of ?1 integrin,CK15,CK19,CEA,CK18.Results The sections were showed tubular or follicular like structures formed with simple or stratified epithelium at 2 and 4 weeks.Keratinized stratified squamous epithelium,sweat glands-like,sebaceous glands-like and hair follicles-like structures were observed at 6 week and 8 week after transplantation.The cells labeled by Hoechst 33342,formed tubular or follicular like structures,expressed?1 integrin,CK15,CK19,CEA and CK18positive respectively at 2 and 4 weeks.The sweat glands-like structure expressed CEA and CK18 positive respectively at 6 and 8 weeks.There were more sebaceous glands-like structures.Conclusion ES cell-derived epidermal stem cells compounded by dermal analogs reconstructed with bone MSCs can differentiate into keratinized stratified squamous epithelium,sweat glands-like,sebaceous glands-like and hair follicles-like structures in hypodermis.

ES CELL-DERIVED EPIDERMAL STEM CELLS FOR TREATMENT OF MICE FULL THICKNESS SKIN DEFECTS / 解剖学报

Objective To provide a new way for treatment of full thickness skin defects by embryonic stem(ES) cell-derived epidermal-like stem cells. Methods Epidermal like stem cells,labeled by Hoechst 33342 and carried by a layer of biomembrane,were transplanted into the defective skin of mice.The differentiation tissue of donor cells was sampled each week.The sections were observed with HE staining,immunohistochemical and di-labeled immunofluorescence methods to test the expression of ?1 integrin,CK15,CK19,CK10,CEA. Results The full thickness skin defects were healed in 2 weeks.The newborn skin was thicker than the normal skin.The basal layer cells proliferated.There were more bulky cellular poles towards dermis.The cells labeled by Hoechst 33342,located in the newborn epidermis and tubular or follicular structures in dermis,expressed ?1 integrin and CK15 positive respectively in the first 3 weeks.There were sweat gland-like,sebaceous gland-like and hair follicle-like structures in the newborn dermis after 4 weeks.Basal cells of keratinized stratified squamous epithelium expressed CK19 and CK10 positive respectively and sweat gland-like structure expressed CEA positive.Conclusion ES cell-derived epidermal stem cells can restore mice full thickness skin defects.There are epidermis,sweat gland-like,sebaceous gland-like and hair follicle-like structures in the newborn skin.