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Cutaneous mastocytosis defines a group of diseases categorized by the existence of augmented numbers of mast cells in the dermis. Cases with cutaneous mastocytosis do not meet diagnostic conditions for systemic mastocytosis and demonstrate no proof of organ participation other than the dermis. This article after mentioning history, prevalence, and classification briefly reviews clinical features and triggers of mast cell activation and lastly discusses the management of cutaneous mastocytosis under the light of current observations in detail. Patients with cutaneous mastocytosis often be influenced by mast cell mediator-linked symptoms, which are often commenced by pressures on the dermal lesions. Management of cutaneous mastocytosis is chiefly built on avoiding triggers of mast cells. The availability of epinephrine autoinjectors in case of severe systemic reactions such as anaphylaxis and utilizing antihistamines when symptoms occur to establish the backbone of treatment in cutaneous mastocytosis cases. [ABSTRACT FROM AUTHOR]

Toads are considered poisonous animals since they have a passive mode of defence relying on cutaneous poison glands, differently from venomous animals who can inject venom in predators/aggressors or prey. Toads of Rhinella marina group are generally large and have a broad distribution in South America, inhabiting a wide range of environments. In this paper, we studied the toads Rhinella icterica from the Atlantic rainforest, and Rhinella jimi from the Brazilian Semiarid Caatinga, analysing aspects of natural history and comparing their skin morphology, the presence of macroglands, their resistance to water loss and rates of water uptake. In periods of extreme drought, R. jimi uses rock cracks as refuges, exposing only the head and regions of accumulation of poison glands. The skin of R. jimi showed higher number of poison glands with hydrophilic content than R. icterica. R. jimi also had a thicker skin, which can be related to its superior resistance to water loss. It also showed high rates of rehydration in association with a peculiar behaviour using the limbs to spread water onto highly glandular skin areas, suggesting that poison glands may also act in water balance in addition to chemical defence. [ABSTRACT FROM AUTHOR]

Ceruminous adenoma is described as a glandular neoplasm of ceruminous glands. It is seen for less than one percent of all external ear tumours. Ceruminous adenoma cases were reported to have recurrence and residual tumour, however there was no malignant transformation known in ceruminous adenoma up to this date. Here, we report a young adult woman with two years history of progressive reduced hearing and tinnitus of the right ear. She was proven to have right moderate conductive hearing loss with pure tone audiometry. There was a cystic mass with serous content arising from the posterior wall of the right ear canal. We proceeded with wide local excision of the mass via trans-canal approach. Ceruminous adenoma was confirmed with histopathology and immunohistochemistry of CK7 staining. The hearing impairment was resolved completely post excision and there was no recurrence of the tumour on one year follow-up. We concluded wide local excision with appropriate margin of the mass is adequate to prevent recurrence in ceruminous adenoma cases. [ABSTRACT FROM AUTHOR]

Abstract Human skin is a region of high metabolic activity where a rich variety of biomarkers are secreted from the stratum corneum. The skin is a constant source of volatile organic compounds (VOCs) derived from skin glands and resident microbiota. Skin VOCs contain the footprints of cellular activities and thus offer unique insights into the intricate processes of cutaneous physiology. This review examines the growing body of research on skin VOC markers as they relate to skin physiology, whereby variations in skin-intrinsic and microbial metabolic processes give rise to unique volatile profiles. Emerging evidence for volatile biomarkers linked to skin perturbations and skin cancer are examined. Microbial-derived VOCs are also investigated as prospective diagnostic markers, and their potential to shape the composition of the local skin microbiota, and consequently cutaneous health, is considered. Finally, a brief outlook on emerging analytical challenges and opportunities for skin VOC-based research and diagnostics is presented. Highlights • Review of research on skin volatiles with a focus on cutaneous health and disease from an analytical chemistry perspective. • Emerging evidence for volatile biomarkers in skin linked to skin perturbations and cancer is examined. • Microbial-derived volatile compounds are reviewed as prospective diagnostic markers. • Analytical challenges and opportunities in the translation of skin volatile markers into clinical diagnostics are discussed. [ABSTRACT FROM AUTHOR]

Background: Cystic fibrosis (CF) is a disorder affecting the respiratory, digestive, reproductive systems and sweat glands. This lethal hereditary disease has known or suspected links to the dysbiosis gut microbiota. High-throughput meta-omics-based approaches may assist in unveiling this complex network of symbiosis modifications. Objectives: The aim of this study was to provide a predictive and functional model of the gut microbiota enterophenotype of pediatric patients affected by CF under clinical stability. Methods: Thirty-one fecal samples were collected from CF patients and healthy children (HC) (age range, 1–6 years) and analysed using targeted-metagenomics and metabolomics to characterize the ecology and metabolism of CF-linked gut microbiota. The multidimensional data were low fused and processed by chemometric classification analysis. Results: The fused metagenomics and metabolomics based gut microbiota profile was characterized by a high abundance of Propionibacterium, Staphylococcus and Clostridiaceae, including Clostridium difficile, and a low abundance of Eggerthella, Eubacterium, Ruminococcus, Dorea, Faecalibacterium prausnitzii, and Lachnospiraceae, associated with overexpression of 4-aminobutyrate (GABA), choline, ethanol, propylbutyrate, and pyridine and low levels of sarcosine, 4-methylphenol, uracil, glucose, acetate, phenol, benzaldehyde, and methylacetate. The CF gut microbiota pattern revealed an enterophenotype intrinsically linked to disease, regardless of age, and with dysbiosis uninduced by reduced pancreatic function and only partially related to oral antibiotic administration or lung colonization/infection. Conclusions: All together, the results obtained suggest that the gut microbiota enterophenotypes of CF, together with endogenous and bacterial CF biomarkers, are direct expression of functional alterations at the intestinal level. Hence, it’s possible to infer that CFTR impairment causes the gut ecosystem imbalance.This new understanding of CF host-gut microbiota interactions may be helpful to rationalize novel clinical interventions to improve the affected children’s nutritional status and intestinal function. [ABSTRACT FROM AUTHOR]

Background: Skin adnexal neoplasms are relatively rare and thus uncommonly encountered in routine pathology practice. Aims: The present study was conducted with the aim to diagnose various skin adnexal tumors on fine-needle aspiration cytology (FNAC) followed by confirmation on histopathology and immunohistochemistry of the excised tumor. Materials and Methods: A total of 11 cases of superficial nodular swellings of the skin were studied over a period of 2 years from 2015 to 2016. FNAC of the swelling was performed and slides were stained with Giemsa stain. Following FNAC, the swelling was excised and subjected to histopathological examination. Immunohistochemistry was performed in some cases for confirmation. Results: It was observed that overall incidence of adnexal tumors (ATs) encountered on FNAC was very low. Typing of ATs on cytology was possible in 82% cases (9/11 cases) with diagnostic accuracy of 88% (8/9 cases). Most common tumor encountered in the study was nodular hidradenoma followed by pilomatrixoma, trichoepithelioma, cylindroma, chondroid syringoma, and trichilemmal cyst. Majority of the cases were benign except one case, which subsequently turned malignant. Conclusion: FNAC is a simple, safe, and cost-effective tool in diagnosing skin ATs. Cytological diagnosis of these lesions is depends on clinical correlation and cytomorphological features. FNAC in lesions suspected to be malignant can guide the surgeon regarding extent of excision. [ABSTRACT FROM AUTHOR]

We have investigated behavioral responses of Rana arvalis Nilss. tadpoles to water carrying various water-soluble chemical stimuli: natural lake water; dechlorinated tap water; water in which tadpoles were kept; water with chemical characteristics of the jellylike remains of their own or other clutches after tadpole hatching; boiled nettle, which was used as food for laboratory animals; and water containing toxins of the skin glands of the common toad Bufo bufo L. The preference for natural water over the tap water was revealed, as well as the fact that naive tadpoles were attracted by the “smell” of nettles and tried to avoid toxins. [ABSTRACT FROM AUTHOR]

Purpose: Comparison of outcomes of localized eyelid sebaceous carcinoma (American Joint Committee on Cancer (AJCC) 8th edition categories T1, T2, T3) following initial management with posterior lamellar resection (PLR) versus (vs) lumpectomy (nonposterior lamellar resection, non-PLR).Methods: This was a retrospective, comparative, interventional case series. Of 54 patients, 26 were managed by PLR and 28 by nonPLR. The main outcome measures were vision loss, orbital exenteration, lymph node metastasis, and remote metastasis were the main outcome measures.Results: A comparison of PLR vs nonPLR revealed no difference in mean age at presentation (70 vs 72 years), race (Caucasian 100% vs nonCaucasian 93%), gender (female 54% vs male 71%), entering mean visual acuity (20/30 vs 20/50), or corneal involvement (23% vs 11%). There was significant difference in clinical features with PLR demonstrating greater mean basal diameter (21 mm vs 12 mm, P = 0.004), and positive margins were seen more frequently in PLR (69% vs 46%). There was significantly less number of surgical procedures in PLR to achieve complete tumor control (1.7 vs 2.5, P = 0.001). Outcomes at mean 55 months follow-up revealed significantly better control with PLR (vs nonPLR) with fewer orbital exenterations (15% vs 43%, P = 0.038), fewer lymph node, and systemic metastases (0% vs 39%, P < 0.001). There was no significant difference in final mean visual acuity (20/60 vs 20/200).Conclusion: For eyelid sebaceous carcinoma, the initial management is critical to the patient's outcome. PLR demonstrates superior outcomes with regard to preservation of visual acuity and avoidance of exenterations, lymph node metastases, and systemic metastases compared to patients managed with other techniques. [ABSTRACT FROM AUTHOR]

Skins of anurans (frogs and toads) are rich sources of bioactive peptides. However, the peptides secreted by the skin glands of Bufo gargarizans , the most common toad in China, remained unexplored to date. Here, a strategy combines LC–MS/MS, RNA sequencing and bioinformation analysis was applied to unravel the peptides in the Bufo gargarizans secretions. Data-dependent LC–MS/MS acquisitions of intact peptides followed by automated chromatographic alignment, De novo analysis, database and homology searches with manual validations showed that the venom is composed by 939 features, with masses ranging from 0.7–4 kDa. These peptides derived from 85 proteins were identified using the PEAKS software with acquired MS and MS/MS spectra of Venenum Bufonis against the house-built protein database using De novo RNA sequencing, while only 23 peptides from 8 proteins were found when searching known amphibian database. Moreover, it was found that many peptides with high abundance in Venenum Bufonis derived from proteolytic processing of a larger precursor protein, named as CL4590. Molecular cloning was applied to validate a short domain of CL4590 to evident the accuracy of these obtained sequences. Although the bioactivities of peptides identified by MS/MS are unknown, the next function annotation showed that they may involve in the cell killing, immune and metabolic process, antioxidant activity and antimicrobial actions. Therefore, the peptidomics analysis on Bufo gargarizans discover abundant novel toad peptides which broaden our horizons on the secretion multiplicity and supplied an assortment of pharmacological candidates. [ABSTRACT FROM AUTHOR]

Abstract: Background: Electrochemotherapy (ECT) is currently used to treat unresectable superficial tumours of different histotypes through the combination of cytotoxic chemotherapy and local application of electric pulses. In 2006, a collaborative project defined the ESOPE (European Standard Operating Procedures of Electrochemotherapy) guidelines to standardize the procedure. The International Network for Sharing Practices of Electrochemotherapy (InspECT) aims to refine the ESOPE and improve clinical practice. Limiting patient exposure to systemic chemotherapy would be advisable to ameliorate ECT safety profile. Objective: The aim of this study was to evaluate the efficacy and toxicity of ECT with reduced chemotherapy dosages. Methods: In a retrospective analysis of a prospectively maintained database (InspECT registry), we evaluated the outcome of patients who received ECT with reduced dosages of bleomycin (7500, 10 000 or 13 500 IU/m2, instead of the standard dose of 15 000 IU/m2). Tumour response in melanoma patients was compared with melanoma patients of the InspECT registry who received the standard dose of bleomycin. Results: We identified 57 patients with 147 tumours (melanoma, 38.6%; squamous cell carcinoma, 22.8%; basal cell carcinoma, 17.5%; breast cancer 7%; Kaposi sarcoma 7%; other histotypes, 7.1%). Per‐tumour complete response (CR) rate at 60 days was 70.1% (partial, 16.3%); per‐patient CR was 57.9% (partial, 21.1%). Local pain was the most frequently reported side‐effect (n = 22 patients [39%]), mostly mild; two patients experienced flu‐like symptoms, one patient nausea. We observed the same CR rate (55%) in patients with melanoma treated by reduced or conventional bleomycin dosages (P = 1.00). Conclusions: Electrochemotherapy performed with reduced bleomycin dosages could be as effective as with currently recommended dose. Patients with impaired renal function or candidate to multiple ECT cycles could benefit from a reduced dose protocol. Our findings need prospective confirmation before being adopted in clinical practice. [ABSTRACT FROM AUTHOR]

Background: Cryopreservation of oocytes is an efficient method of fertility preservation (FP) that can be applied in women suffering from gynecologic conditions that menace their reproductive future. Collection of oocytes becomes challenging in some scenarios, like the possibility of an ovarian cancer, the "ex-vivo" harvest of oocytes for FP, aspirating follicles directly from the ovarian specimen already excised by laparotomy or laparoscopy and it is an option for these cases. Case Presentation: In the present case report, the case of a patient with an adnexal mass suspected to be a recurrent teratoma was described who referred to our Assisted Reproduction Unit in Hospital Quironsalud Malaga for FP counseling. After controlled ovarian hyperstimulation, followed by laparoscopic abdominal examination and oophorectomy, an ex-vivo follicular aspiration for oocyte retrieval was performed on the specimen, using a standard ultrasound-guided procedure to ease and improve the process. All the follicles were aspirated and 5 metaphase II oocytes were obtained. Conclusion: This is to our knowledge, the first communication describing the ex-vivo ovarian aspiration of mature oocytes for FP using standard ultrasound guidance. Although this ultrasound guidance is not completely necessary, as other authors demonstrated previously, such a procedure permitted an easy and complete harvest of oocytes in a rare tumor with bizarre cystic formations, which made follicle recognition very difficult. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Upper extremity lymphedema and cutaneous spread are atypical behavior of prostate disease and should be kept in the differential for selected patients. This presentation in these patients may be underdiagnosed and potentially an ominous sign. Our case adds to our continued learning of possible prostate malignancy behavior. [ABSTRACT FROM AUTHOR]

Abstract: Background: Sebaceous nevus is a congenital malformation of the skin that usually occurs on the scalp or face. Syndromic forms do rarely exist with associated cerebral and ocular malformations. The skin lesions are pale at birth and become irregular by puberty. In the adult patient, tumors (usually benign) develop from sebaceous nevus. Their surgical excision during childhood can give a better result in terms of the definitive scar. Objectives: The aim of this study is to analyze our cases of syndromic sebaceous hamartoma, perform a review of the existing literature, and propose guidelines for the therapeutic plan. Methods: This is a retrospective study reviewing the cases of syndromic sebaceous nevus treated in the Department of Orthopedic Plastic Pediatric Surgery in Montpellier, France, and the Department of Pediatric Surgery in Lausanne, Switzerland, between 1994 and 2016. Results: The files of six patients with syndromic sebaceous nevus were analyzed. The average age at the first consultation was 4 months. The location was craniofacial in all cases. Cerebral radiological imaging was performed on all patients; two showed abnormal findings. Four patients underwent ophthalmic examination, which all revealed abnormalities. Three patients had other associated malformations. Three patients presented with epilepsy or learning difficulties in the course of follow‐up. Conclusion: All patients presenting with extensive sebaceous nevus of the craniofacial region should benefit from cerebral imagery and ophthalmic examination since there is a very high probability of associated abnormalities. The developmental problems encountered could not be definitively associated with the skin malformations. [ABSTRACT FROM AUTHOR]

Background To determine in vivo effects of CFTR modulators on mutation S945L. Methods We measured effects of CFTR modulators on CFTR-dependent sweating (‘C-sweat’) in two pancreatic sufficient cystic fibrosis (CF) subjects. S1 (S945L/G542X) took ivacaftor and S2 (S945L/F508del) took ivacaftor + tezacaftor. Sweating was stimulated pharmacologically to produce sequentially both CFTR-independent (methacholine stimulated) M-sweat and C-sweat; and the ratio of these was compared. Sweat secretion was measured with two methods: real time secretory rate quantitative recording and by optically measuring the growth of sweat bubbles under oil from multiple identified glands. Results Using the quantitative recorder, we saw zero C-sweat secretion off-drug, but when on-drug the C-sweat responses for both subjects were comparable to those seen in carriers. The on-drug response was further quantified using the sweat bubble method. Each subject again showed robust C-sweat responses, with C-sweat/M-sweat ratios ~ half of the ratio determined for a cohort of 40 controls tested under identical conditions. Conclusion These in vivo results, consistent with prior in vitro findings, indicate that the drug treatments restore near-normal function to S945L-CFTR, and support the use of ivacaftor as a treatment for CF patients who carry this allele. [ABSTRACT FROM AUTHOR]

Abstract: Electrochemotherapy (EQT) is a local cancer treatment well established to cutaneous and subcutaneous tumors. Electric fields are applied to biological tissue in order to improve membrane permeability for cytotoxic drugs. This phenomenon is called electroporation or electropermeabilization. Studies have reported that tissue conductivity is electric field dependent. Electroporation numerical models of biological tissues are essential in treatment planning. Tumors of the mouth are very common in dogs. Inadequate EQT treatment of oral tumor may be caused by significant anatomic variations between dogs and tumor position. Numerical models of oral mucosa and tumor allow the treatment planning and optimization of electrodes for each patient. In this work, oral mucosa conductivity during electroporation was characterized by measuring applied voltage and current of ex vivo rats. This electroporation model was used with a spontaneous canine oral melanoma. The model outcomes of oral tumor EQT is applied in different parts of the oral cavity including near bones and the hard palate. The numerical modeling for treatment planning will help the development of new electrodes and increase the EQT effectiveness. [ABSTRACT FROM AUTHOR]

Background Demographic, clinical, and morphological characteristics of cutaneous melanoma are unknown for the Colombian population. We aim to provide these characteristics as well as population-based incidence and survival data. Methods All patients with an invasive cutaneous melanoma diagnosed in the period 2000-2009 registered in the population-based cancer registry of the metropolitan area of Bucaramanga were included for analysis ( n = 169). Age-standardized incidence rates were calculated and melanoma-specific and overall survival estimated with follow-up until June 9, 2016, using Kaplan-Meier methodology, stratifying for gender, anatomical localization, and type of affiliation to the social security system. Results The age-standardized melanoma incidence rate was 1.7 per 100,000, with lower limbs being the most affected body sites (42.6% of all melanomas). A high proportion of melanomas presented on the plants or palms (16%) and under the nails (7.1%); at least 24.3% of melanomas were ulcerated, and 21.1% had a Breslow thickness more than 2 mm. Melanoma-specific 5-year survival was 79.3%, with worst survival for melanomas localized on the plants (64.6%) and subungual areas (55.6%). Affiliation to the subsidized type of affiliation to the social security system was statistically significantly ( P = 0.003) associated with poorer survival (68.8%) compared to the special regimes (95.8%). Conclusions Melanoma is a relatively rare cancer in Colombia with mainly the acral sites, high proportion of thick and ulcerated melanomas, and relatively poor survival being distinct features, indicating the need for tailor-made primary and secondary prevention strategies. Better training of pathologists in the difficult field of melanoma would improve precision of available data. [ABSTRACT FROM AUTHOR]

This article provides an overview of selected genetic skin conditions where multiple inherited cutaneous tumours are a central feature. Skin tumours that arise from skin structures such as hair, sweat glands and sebaceous glands are called skin appendage tumours. These tumours are uncommon, but can have important implications for patient care. Certain appendageal tumours, particularly when multiple lesions are seen, may indicate an underlying genetic condition. These tumours may not display clinical features that allow a secure diagnosis to be made, necessitating biopsy and dermatopathological assessment. Coupled with robust clinical assessment, biopsy findings can guide genetic testing as, increasingly, the causative genes are known for these conditions. Here we review illustrative examples of appendageal tumours and relevant advances made in genetic discovery, and suggest when referral to a geneticist may need to be considered. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the characteristics of patients who underwent surgery due to the presence of a large pelvic-abdominal mass over a 5-year period in a university clinic. Material and Methods: Among 3476 gynecologic operations, intraoperative findings were evaluated retrospectively. Uterine and/or adnexal masses smaller than 20 cm were excluded to refine "large" tumors and 74 patients with large tumors were enrolled in the study group. Demographic characteristics, intraoperative findings, and results of histopathologic examinations were recorded. Moreover, preoperative and intraoperative findings were compared among tumors with adnexal origin according to their final histopathologic results. Results: The mean age of the patients was 46 years. The most common symptom was abdominal pain, as recorded in 38 (51.4%) patients. Among all patients, 31 (41.9%) had coexisting illness and 13 (17.6%) had a history of surgery. The mean tumor diameter was 25.9±8.6 cm (20-60) and 60 (78.9%) tumors were of adnexal origin. The ratios of malignancy for large adnexal and uterine tumors were 34.4% and 12.5%, respectively. When the large adnexal tumors were re-evaluated, the mean cancer antigen (CA) 125 level was significantly higher, and ascites was more frequently detected in malignant tumors (p<0.01) then in benign and borderline tumors. Conclusion: Benign and borderline tumors are more common among large abdominopelvic masses, although the presence of ascites and elevated CA 125 may present malignancy in large gynecologic tumors. Further studies with larger sample sizes are needed to define the characteristics of large tumors and their malignant potentials. [ABSTRACT FROM AUTHOR]

Canine melanoma ( CMM) more commonly affects the oral mucosa and the cutis. CMM shares several features with human melanomas ( HMM), included resistance to a broad variety of antineoplastic chemotherapy agents. P-glycoprotein 1 (Pgp) expression is a well-recognized feature of multi-drug resistance and the purpose of this study was to investigate its expression in treatment naïve CMM. We also investigated Pgp association with tumour location and histological features. Histology records of CMM were retrieved, including patients from 2012-2014. Twenty-five cases of CMM were included in this study. Results revealed that Pgp is expressed in CMM and oral tumours were more likely to have a membranous Pgp expression (100%) than cutaneous tumours (66.6%) ( P = 0.010). Cytoplasmic and nuclear Pgp expression could also be identified. Results of this study bring useful data that help in understanding one of the possible mechanisms responsible of intrinsic chemotherapy resistance in canine CMM. [ABSTRACT FROM AUTHOR]

Cutaneous larva migrans (CLM) is a parasitic skin disease caused by the accidental percutaneous penetration and subsequent intraepidermal migration of larvae of various nematode parasites of the hookworm family. The hookworms responsible for CLM are spread worldwide, but the infection occurs mostly in tropical and subtropical climates. Nowadays, because of ever increasing foreign travel, the disease is no longer confined to these areas. Moreover, a significant increase of autochthonous cases in southern Europe has been observed in recent years. We report two new patients from Serbia who acquired CLM, none of them having traveled abroad. As the recommendations for the treatment of CLM are not uniform, two different treatment modalities were applied in these patients, both with an excellent response. [ABSTRACT FROM AUTHOR]

Aims. To assess whether replacing CA125 with HE4 in the classical formulas of risk of malignancy indices (RMIs) can improve diagnostic performance. Methods. For each of 312 patients with an adnexal mass, classical RMIs 1–4 were computed based on ultrasound score, menopausal status, and serum CA125 levels. Additionally, modified RMIs (mRMIs) 1–4 were recalculated by replacing CA125 with HE4. Results. Malignant pathology was diagnosed in 52 patients (16.67%). There was no significant difference in diagnostic performance (area under the receiver operating characteristic curve [AUC]) between each classical RMI and its corresponding mRMI. In the entire sample, the AUC was 0.899, 0.900, 0.895, and 0.908 for classical RMIs 1–4 compared to 0.903, 0.929, 0.930, and 0.931 for mRMIs 1–4. In premenopausal patients, the AUC was 0.818, 0.798, 0.795, and 0.802 for classical RMIs 1–4 compared to 0.839, 0.875, 0.876, and 0.856 for mRMIs 1–4. In postmenopausal patients, the AUC was 0.906, 0.895, 0.896, and 0.906 for classical RMIs 1–4 compared to 0.907, 0.923, 0.924, and 0.930 for mRMI 1–4. Conclusions. Use of HE4 instead of CA125 did not significantly improve diagnostic performance of RMIs 1–4 in patients with an adnexal mass. [ABSTRACT FROM AUTHOR]

Purpose: Overexpression of the inhibitors of apoptosis proteins have been demonstrated in a variety and of solid tumors including melanomas and nonmelanomas skin cancers. X-linked inhibitor of apoptosis protein (XIAP) is an inhibitor of apoptosis which prevents apoptosis by inhibiting caspases 9, 7, and 3. The prognostic value of XIAP in sebaceous gland carcinoma (SGC) remains unexplored.Methods: The immunohistochemical expression of XIAP was evaluated in 29 SGC cases.Results: The cytoplasmic overexpression of XIAP was detected in 62% SGC cases. XIAP expression was found to be significantly associated with advanced age, large tumor size, and with reduced disease-free survival (P = 0.0174). XIAP expression and advance tumor Grade III emerged as significant risk factors on univariate analysis. On stepwise multivariate analysis, both increased cytoplasmic XIAP expression and high tumor grade were found to be significantly associated with recurrence. Patients with low XIAP immunoexpression had a longer disease-specific survival than those with high expression in the 5-year follow-up.Conclusion: The present study demonstrates at the immunohistochemical level that XIAP is overexpressed in SGC and that high expression could be of biological significance in the development of eyelid SGC. Our finding suggests that up-regulation of XIAP may aggravate tumor metastasis in SGC. [ABSTRACT FROM AUTHOR]

Dysregulated human eccrine sweat glands can negatively impact the quality-of-life of people suffering from disorders like hyperhidrosis. Inability of sweating can even result in serious health effects in humans affected by anhidrosis. The underlying mechanisms must be elucidated and a reliable in vitro test system for drug screening must be developed. Here we describe a novel organotypic three-dimensional (3D) sweat gland model made of primary human eccrine sweat gland cells. Initial experiments revealed that eccrine sweat gland cells in a two-dimensional (2D) culture lose typical physiological markers. To resemble the in vivo situation as close as possible, we applied the hanging drop cultivation technology regaining most of the markers when cultured in its natural spherical environment. To compare the organotypic 3D sweat gland model versus human sweat glands in vivo, we compared markers relevant for the eccrine sweat gland using transcriptomic and proteomic analysis. Comparing the marker profile, a high in vitro-in vivo correlation was shown. Carcinoembryonic antigen-related cell adhesion molecule 5 (CEACAM5), muscarinic acetylcholine receptor M3 (CHRM3), Na+-K+-Cl- cotransporter 1 (NKCC1), calcium-activated chloride channel anoctamin-1 (ANO1/TMEM16A), and aquaporin-5 (AQP5) are found at significant expression levels in the 3D model. Moreover, cholinergic stimulation with acetylcholine or pilocarpine leads to calcium influx monitored in a calcium flux assay. Cholinergic stimulation cannot be achieved with the sweat gland cell line NCL-SG3 used as a sweat gland model system. Our results show clear benefits of the organotypic 3D sweat gland model versus 2D cultures in terms of the expression of essential eccrine sweat gland key regulators and in the physiological response to stimulation. Taken together, this novel organotypic 3D sweat gland model shows a good in vitro-in vivo correlation and is an appropriate alternative for screening of potential bioactives regulating the sweat mechanism. [ABSTRACT FROM AUTHOR]

The current study investigated the effects of a low back pain (LBP) vibration modality on trunk motor control. Trunk repositioning error and responses to a sudden loading trunk perturbation were evaluated pre- and post-vibration (15min vibration exposure while sitting on a standard chair) as well as when concurrent cutaneous low back vibration was applied. Only minor effects were observed post-vibration when compared to pre-vibration. However, when vibration was applied at the same time as the sudden trunk perturbations, lumbar erector spinae and external oblique muscles were significantly more delayed in activating following the perturbation. In addition, the resting muscle activation prior to the trunk perturbation was higher in both the back extensor and abdominal muscles when concurrent vibration was applied. These findings suggest that cutaneous low back vibration significantly alters motor control responses and this should be considered before implementing cutaneous vibration as a low back pain management strategy. [ABSTRACT FROM AUTHOR]

Background: Skin adnexal tumors (SAT) encompass wide spectrum of benign and malignant tumors that differentiate toward one or more adnexal structures found in normal skin. Overall incidence of SATs is low yet they can be challenging to diagnose. Aims: The aim of this study is to study the spectrum and microscopic features of SATs. Materials and Methods: It was a retrospective cross-sectional, descriptive study conducted over a period of 3 years. Formalin fixed, paraffin-embedded sections were stained with hematoxylin and eosin for histopathological analysis. Results: Out of the total 34,400 biopsies, 110 cases were diagnosed as SATs comprising 39.09% of tumors with follicular differentiation followed by tumors showing sweat gland differentiation (37.27%), and sebaceous differentiation (23.63%). The age ranged from 5 years to 85 years and male:female ratio was 1.03:1. Most of the tumors were benign (82.73%) while only 17.27% were malignant. Pilomatricoma (28.2%) was the most common benign tumor while sebaceous carcinoma (11.8%) was the most common malignant tumor. Conclusion: Architectural features are of great importance in differentiating benign tumors from malignant. [ABSTRACT FROM AUTHOR]

Parents in many taxa, including insects, molluscs, fish, snakes, and amphibians provision chemical defences, such as peptides, steroids, or alkaloids to their offspring to reduce the risk of predation. In most cases, those defences are transferred to offspring in the egg and gradually diminish throughout the larval period. Adult poison frogs sequester alkaloid-based defences from arthropod prey in granular skin glands. In at least one poison frog, Oophaga pumilio, mother frogs intermittently feed tadpoles until metamorphosis with nutritive eggs containing those alkaloid-based defences. However, alkaloids are not detected in tadpoles until they reach the middle stages of larval development. Here, we investigate the histology of a developmental series of O. pumilio tadpoles to determine whether their ontogenetic alkaloid profile coincides with granular gland development. Our findings suggest that alkaloid sequestration in tadpoles is delineated by the differentiation of rudimentary granular skin glands in epithelial tissue. The timing of differentiation of granular glands in this species coincides with other anurans. Thus, provisioning of chemical defences to offspring is likely constrained by developmental timing of derived structures that can effectively store those toxic or noxious compounds. [ABSTRACT FROM AUTHOR]

Background: Adnexal tumors of the skin are a large and diverse group of benign and malignant neoplasms, which exhibit morphological differentiation toward one of the different types of adnexal epithelium present in normal skin and they pose a diagnostic challenge. The purpose of this study is to share our experience with these rare but aggressive tumors at a tertiary care cancer hospital in a developing country. A retrospective review of 11 patients diagnosed with rare adnexal tumors and their variants from January 2005 to December 2014, treated either surgically or non-surgically, was performed to describe the clinicopathological characteristics and outcome of the disease.Case Presentation: A total of 11 patients were diagnosed with adnexal carcinoma and its variants: a 34-year-old Sindhi man, a 59-year-old Punjabi woman, a 32-year-old woman from Khyber Pakhtunkhwa, a 43-year-old Punjabi woman, a 64-year-old Punjabi man, a 51-year-old man from Khyber Pakhtunkhwa, a 51-year-old Punjabi woman, a 74-year-old Punjabi woman, a 75-year-old Punjabi man, a 61-year-old man from Khyber Pakhtunkhwa, and a 53-year-old man from Khyber Pakhtunkhwa. The male to female ratio was 1.2:1. The histological variations were sebaceous differentiation (n = 4), microcystic adnexal carcinoma (n = 4), trichilemmal carcinoma (n = 1), pilomatrix carcinoma (n = 1), and hidradenocarcinoma (n = 1). The mean age at presentation was 54 years (range 32 to 75). The primary subsite of involvement was the scalp in nine patients followed by eyelids in two patients. Surgery was the primary treatment modality in almost all patients; postoperative radiotherapy (PORT) was offered to eight patients. The median dose of radiation was 45 Gy to the primary site. Indications for radiotherapy included close margins (n = 2), positive margins (n = 1), high grade histology (n = 4), and multifocal disease (n = 1). On follow-up, two patients presented with local, one regional and two patients developed distant metastasis.Conclusions: Adnexal carcinomas are rare tumors with diverse histological patterns and a tendency for locoregional and distant metastasis. Surgery should be the mainstay of treatment reserving radiotherapy for adjuvant, palliative, and re-treatment scenarios. [ABSTRACT FROM AUTHOR]

Background: Ovarian cancer is one of the highest mortality cancers in gynaecology. Discrimination of benign masses from malignant ones may sometimes become a challenge for the clinician since there is not a reliable tumour marker, thus some unnecessary, highly morbid operations can be performed. Aims: To explore the efficacy of human epididymis 4 (HE 4) and cancer antigen 125 (CA 125) markers in differentiating malignant and benign pelvic masses of ovarian origin and to identify the cut-off points for those markers. Study Design: Prospective study. Methods: Fifty-one patients who were diagnosed and planned to undergo surgery for ovarian mass between June 2008 and December 2008 were enrolled into this study. Preoperative venous blood samples were taken and frozen for marker investigation and final diagnoses were concluded by histopathological examination. After recruitment of all cases CA 125 and HE 4 levels were evaluated. Results: The statistical analysis did not indicate any statistically significant difference between the CA 125 levels of the patients with malignant and benign adnexal masses (p=0.105). The HE 4 levels of the patients with malignant adnexal masses were higher at a statistically significant level compared to the patients with benign adnexal masses (p=0.002). For HE 4 tumour marker and at the cut-off point of >25 pM, sensitivity was 1, specificity 0.40, positive cut-off value 0.19, negative cut-off value 1, accuracy 0.47 and positive likelihood ratio 1.65. Conclusion: Human epididymis 4 is a better diagnostic tool than CA 125 in benign-malignant discrimination of adnexal masses. The cut-off value of 25 pmol/L for human epididymis 4 will contribute to providing proper guidance to patients with adnexal masses and applying the proper treatment method. [ABSTRACT FROM AUTHOR]

We review and emphasize the importance of gynecologic ultrasound scan for the preoperative evaluation of adnexal masses. Trans vaginal ultrasound performed by a trained clinician has a good sensitivity and specificity for discriminating benign and malignant adnexal masses. In conjunction with a carefully obtained history, assessment of risk factors, a focused physical examination and serum markers, the information obtained by a gynecologic ultrasound evaluation can assist the clinician in the diagnosis and treatment of adnexal masses. [ABSTRACT FROM AUTHOR]

Aim The aim of the present study was to evaluate the usefulness of nestin as a discriminative marker between benign and malignant ovarian tumors. Methods During the 1 year from January 2015 through December 2015, a nonconsecutive series of 80 patients (40 malignant, 40 benign) who underwent surgery for an adnexal mass were enrolled in the study. Intraoperative frozen section evaluation was performed if there was a suspicion in diagnosis. Statistical analyses were performed using spss ver. 16.0, while clinicopathological variables, including the categorical data, were analyzed using the χ2-test or Fisher's exact test. A P-value < 0.05 was defined as statistically significant. Results Preoperative serum carbohydrate antigen (CA)-125, CA-15-3, and nestin levels were significantly higher in the malignant group compared to patients with benign ovarian tumors ( P < 0.001, respectively). Serum nestin levels did not differ significantly on the basis of histologic subtypes. Serum nestin levels had specificity of 89.7%, which demonstrates nestin's sufficiency to distinguish benign from malignant epithelial ovarian tumors. The positive likelihood ratio of nestin was found to be superior to that of CA-125 and CA-15-3. Conclusion The results obtained from our study suggest that measurement of nestin level, alongside physical examination, transvaginal ultrasound, and serum CA-125 and CA-15-3 levels, can help differentiate benign ovarian tumors from malignant epithelial ovarian tumors. The findings of our study need to be supported with additional studies. [ABSTRACT FROM AUTHOR]

Background: Bell's palsy is the most common cause of facial paralysis worldwide and the most common disorder of the cranial nerves. It is a diagnosis of exclusion, accounting for 60-75% of all acquired peripheral facial nerve palsies. Our case shows the first case of a microcystic adnexal carcinoma-like squamous cell carcinoma as a cause of facial nerve palsy. Case presentation: The patient, a 70-year-old Caucasian male, experienced subsequent functional impairment of the trigeminal and the glossopharyngeal nerve about 1 1/2 years after refractory facial nerve palsy. An extensive clinical work-up and tissue biopsy of the surrounding parotid gland tissue was not able to determine the cause of the paralysis. Primary infiltration of the facial nerve with subsequent spreading to the trigeminal and glossopharyngeal nerve via neuroanastomoses was suspected. After discussing options with the patient, the main stem of the facial nerve was resected to ascertain the diagnosis of MAC-like squamous cell carcinoma, and radiochemotherapy was subsequently started. Conclusion: This case report shows that even rare neoplastic etiologies should be considered as a cause of refractory facial nerve palsy and that it is necessary to perform an extended diagnostic work-up to ascertain the diagnosis. This includes high-resolution MRI imaging and, as perilesional parotid biopsies might be inadequate for rare cases like ours, consideration of a direct nerve biopsy to establish the right diagnosis. [ABSTRACT FROM AUTHOR]

Background: Facial wounds are challenging for dermatologic surgeons, particularly traumatic facial wounds, because they can yield disfiguring scars. To obtain good results, narrow needles and sutures are needed. Hair filaments have a very small diameter (0.06-0.1 mm) and could serve as suture threads for facial wounds. Objective: To determine the aesthetic outcomes by using autologous hair to suture facial wounds. Patients and Methods: This case series study examined the aesthetic outcomes of all consecutive female patients with traumatic facial wounds who underwent autologous hair-based stitching in 2009-2016. Autologous hair ampouleswere generated from an insulin needle. Micro instruments were used for wound stitching. Results: In total, 54 females (mean age, 10.8; range, 3-45) years had 56 traumatic wounds. Mean wound length was 3.6 (range, 1-12) cm. Injury depth varied from cutaneous-only to muscle involvement. Suturing yielded good edge coaptation, nice healing, and excellent aesthetic outcomes; the scarswere often scarcely visible. Suturemarkswere not detected. Cutaneous reactions did not occur. Conclusion: Autologous hair can serve as a thread for closing facial wounds. It is lowcost and thus suitable in settings characterized by facility and equipment limitations. It is also suitable for the battlefield. [ABSTRACT FROM AUTHOR]

Objective: Eyelid carcinomas are rare, and the management strategy of regional lymph node metastasis linked to eyelid carcinomas has not been standardized to date. The aim of the present study was to analyze the patterns of regional metastasis and to assess the optimal extent of surgical treatment for lymph node metastasis of eyelid carcinoma.Methods: This study was a retrospective review of patient data from a single institution. From a series of 268 eyelid carcinomas, we selected the 21 patients with lymph node metastasis, and we analyzed the patterns of lymphatic spread, approach to treatment and outcomes.Results: The most common histological type of eyelid carcinoma with regional metastasis was sebaceous carcinoma (17/21, 81.0%). Submandibular area metastases were seen only in the patients with the primary tumor originating in the medial half of the eyelid, but parotid area metastases were seen in both the patients whose tumors had a medial-half origin and those with a lateral-half origin. Although 11 of the 16 patients with parotid-area metastases underwent a tumorectomy or superficial parotidectomy (which resulted in four cases of recurrence in the parotid area), none of the five patients who underwent a total parotidectomy developed parotid-area recurrence. The incidence of regional recurrence of the patients who received adjuvant radiotherapy (14.3%) was lower than that of the patients without adjuvant radiotherapy (57.1%).Conclusion: Continued surveillance and optimal management of regional lymph node metastases are important for the control and survival of eyelid carcinomas. [ABSTRACT FROM AUTHOR]

The skin glands and cloacal morphology of the Korean crevice salamander, Karsenia koreana, were similar to those of other plethodontids. The skin contained mucous, granular, and modified granular glands in varying frequencies and sizes. Males had sexually dimorphic glands in the skin of the chin (mental glands) and the dorsal tail base (caudal courtship glands). On the ventral surface of the tail base, modified granular glands were sexually dimorphic in size, with male glands larger than those in females. The cloacal glands in males, as in other plethodontids, consisted of four eosinophilic gland clusters (dorsal pelvic glands, lateral pelvic glands, caudal pelvic glands, and vent glands) and three basophilic glands (anterior ventral glands, posterior ventral glands, and Kingsbury's glands). In females, the only cloacal gland was the spermatheca, which, as in other plethodontids, was a compound tubulo-alveolar gland in the roof of the cloaca. [ABSTRACT FROM AUTHOR]

A Wolffian tumor is a rare tumor arising from the remnants of the mesonephric duct. Herein, we report two cases. A 61-year-old woman presented with swollen left adnexa. On T2 weighted magnetic resonance imaging (MRI), a 4.4 cm solid pelvic mass showed slightly high signal intensity, with a low-signal-intensity rim. Microscopically, cuboidal cells with bland nuclei were arranged in a dense tubular form. A 40-year-old woman complained of lower abdominal pain. On T2 weighted MRI, a 6.0 cm solid mass with a low-signal-intensity rim was found. The pathology of the resected tumor was similar to that of case 1. Both cases were diagnosed as Wolffian tumor. The rims of the tumors in both cases were composed of eosinophilic spindle-shaped cells immunoreactive to alpha-smooth muscle actin and desmin, identical to the smooth muscle cells surrounding the mesonephric duct remnants. MRI findings reflecting smooth muscle rims might contribute to an accurate preoperative diagnosis of this rare tumor. [ABSTRACT FROM AUTHOR]

Polymorphous sweat gland carcinoma ( PSGC) is a rare adnexal neoplasm with characteristic variegated histopathologic findings and low-grade clinical behavior. First described in 1994, only 11 cases have been reported in the literature. It is named for the multiplicity of architectural patterns that may be present: solid, tubular, trabecular, pseudopapillary and cylindromatous. Owing to the multiple architectural patterns, the differential diagnosis is broad, including metastatic adenocarcinoma and other adnexal neoplasms with ductular differentiation. We present two new cases of PSGC and review the literature on this rare tumor. [ABSTRACT FROM AUTHOR]

We examined skin blood flow (SkBF) and vasomotion in the forearm and leg using laser-Doppler fluxmetry (LDF) and spectral analysis to investigate endothelial, sympathetic, and myogenic activities in response to slow (0.1 °C·10 s − 1 ) and fast (0.5 °C·10 s − 1 ) local heating. At 33 °C (thermoneutral) endothelial activity was higher in the legs than the forearms ( P ≤ 0.02). Fast-heating increased SkBF more than slow heating ( P = 0.037 forearm; P = 0.002 leg). At onset of 42 °C, endothelial ( P = 0.043 forearm; P = 0.48 leg) activity increased in both regions during the fast-heating protocol. Following prolonged heating (42 °C) endothelial activity was higher in both the forearm ( P = 0.002) and leg ( P < 0.001) following fast-heating. These results confirm regional differences in the response to local heating and suggest that the greater increase in SkBF in response to fast local heating is initially due to increased endothelial and sympathetic activity. Furthermore, with sustained local skin heating, greater vasodilatation was observed with fast heating compared to slow heating. These data indicate that this difference is due to greater endothelial activity following fast heating compared to slow heating, suggesting that the rate of skin heating may alter the mechanisms contributing to cutaneous vasodilatation. [ABSTRACT FROM AUTHOR]

Toad glandular secretions and skin extractions contain numerous natural agents which may provide unique resources for novel drug development. Especially the skin-parotoid gland secretions of toads from genus Bufo contain as many as 86 different types of active compounds, each with the potential of becoming a potent drug. In the present study, crude skin-parotoid gland secretions from Bufo bufo , Bufo verrucosissimus and Bufotes variabilis from Turkey were screened against various cancer cells together with normal cells using MTT assay. Furthermore, the antimicrobial properties of skin secretions were tested on selected bacterial and fungal species for assessing the possible medical applications. Antimicrobial activity of skin secretions was studied by determining minimal inhibitory concentration (MIC) in broth dilution method. Hemolytic activity of each skin-secretion was also estimated for evaluating pharmaceutical potential. Both skin-parotoid gland secretions showed high cytotoxic effect on all cancerous and non-cancerous cell lines with IC 50 values varying between <0.1 μg/ml and 6.02 μg/ml. MIC results of antimicrobial activity tests were found to be between 3.9 μg/ml and 250 μg/ml. No hemolytic activities on rabbit red blood cells at concentrations between 0.5 μg/ml and 50 μg/ml were observed. In conclusion, skin-parotoid secretions of bufonid toads might be remarkable candidates for anti-cancer and antimicrobial agents without hemolytic activities. [ABSTRACT FROM AUTHOR]

The vicuña ( Vicugna vicugna ) is the smallest member of the Camelidae with one of the most valuable fibers in the world. However, little is known of the structural basis for this fiber or of the skin glands used in chemical communication in this species. The objective of this study was to examine the histological structure of the skin and hair follicles and the cutaneous glands. Skin samples from the fleece, belly and legs and glandular areas – metatarsal, caudal, anal and interdigital areas – were obtained from animals accidentally killed in captive captures at a government camelid ranch in Jujuy, Argentina. These samples were studied with histological techniques for light and electron microscopy to examine follicular structure and density in fleece compared to belly and leg areas. Glandular areas were similarly studied to determine type and density of skin glands. Compound hair follicles were found with an extremely high density of secondary hairs in relation to primary hairs (S:P ratio) in fleece (42 ± 2, mean ± standard error) and a significantly lesser density (p < 0.001) in belly and legs (12.0 ± 0.7). Follicle and hair diameters from the two areas were also significantly different with the fleece areas having smaller diameters. Secondary hair diameters in fleece were similar to previously published values (13.2 ± 0.3 μm). With respect to the glandular areas, the metatarsal glands had a striking appearance with an epithelium resembling a hollow honeycomb and a dermis showing a great deal of glandular tissue (simple tubular glands) with excretory ducts. Large sebaceous glands were found in the ventral region of the tail and eccrine sudoriferous glands in the interdigital region. The possible importance of these glands in chemical communication is discussed. [ABSTRACT FROM AUTHOR]

Objective: Cutaneous melanoma is the most hazardous malignancy of skin cancer with a high mortality rate. It has been reported that cancer stem cells (CSCs) are responsible for malignancy in most of cancers including melanoma. The aim of this study is to compare two common methods for melanoma stem cell enriching; isolating based on the CD133 cell surface marker and spheroid cell culture. Materials and Methods: In this experimental study, melanoma stem cells were enriched by fluorescence activated cell sorting (FACS) based on the CD133 protein expression and spheroid culture of D10 melanoma cell line,. To determine stemness features, the mRNA expression analysis of ABCG2, c-MYC, NESTIN, OCT4-A and -B genes as well as colony and spheroid formation assays were utilized in unsorted CD133+, CD133 and spheroid cells. Significant differences of the two experimental groups were compared using student's t tests and a two-tailed value of P<0.05 was statistically considered as a significant threshold. Results: Our results demonstrated that spheroid cells had more colony and spheroid forming ability, rather than CD133+ cells and the other groups. Moreover, melanospheres expressed higher mRNA expression level of ABCG2, c-MYC, NESTIN and OCT4-A compared to other groups (P<0.05). Conclusion: Although CD133+ derived melanoma cells represented stemness features, our findings demonstrated that spheroid culture could be more effective method to enrich melanoma stem cells. [ABSTRACT FROM AUTHOR]

Background Eccrine sweat secretion is of central importance for control of body temperature. Although the incidence of sweat gland dysfunction might appear of minor importance, it can be a real concern for people with either hypohidrosis or hyperhidrosis. However, sweat gland function remains relatively poorly explored. Objectives To investigate the function of single human sweat glands. Methods We describe a new approach for noninvasive imaging of single sweat gland activity in human palms in vivo up to a depth of 100 μm, based on nonlinear two-photon excited autofluorescence ( TPEF) and coherent anti-Stokes Raman scattering ( CARS). Results These techniques appear to be useful compared with approaches already described for imaging single sweat gland activity, as they allow better three-dimensional spatial resolution of sweat pore inner morphology and real-time monitoring of individual sweat events. By filling the sweat pore with oil and tuning the CARS contrast at 2845 cm−1, we imaged the ejection of sweat droplets from a single sweat gland when oil is pushed out by sweat flow. On average, sweat events lasted for about 30 s every 3 min under the conditions studied. On the other hand, about 20% of sweat glands were found inactive. TPEF and CARS were also used to study, at the single pore level, the antiperspirant action of aluminium chlorohydrate ( ACH) and to reveal, for the first time in vivo, the formation of a plug at the pore entrance, in agreement with reported ACH antiperspirant mechanisms. Conclusions Although data were acquired on human palms, these techniques show great promise for a better understanding of sweat secretion physiology and should be helpful to improve the efficacy of antiperspirant formulations. [ABSTRACT FROM AUTHOR]

Buschke-Ollendorff syndrome (BOS) is a rare, often benign, autosomal skin disorder. BOS commonly presents with nontender connective tissue naevi and sclerotic bony lesions (osteopoikilosis [OPK]). Herein, we summarize the presenting features of BOS and potential associations by conducting a systematic review of the literature and summarizing a cohort seen at the Hospital for Sick Children (HSC), Toronto, Canada. PubMed was searched using the following terms: 'BOS'; 'dermatofibrosis lenticularis'; 'OPK'; 'LEMD3'; 'elastoma'; 'collagenoma'. Only case reports were included, without date or language restrictions. Cases were further narrowed to those where patients or their families had a combination of skin and bony lesions, or a positive genetic test. Data were summarized using frequencies. In total, 594 reports were discovered, of which 546 (92%) were excluded. The remaining 48 accounted for 164 cases. Skin lesions were noted in 24% of cases and bony lesions in 20%, while 54% of patients had both. In 1% of cases the diagnosis was made on genetic testing alone. A family history was noted in 92% of cases. All patients with spinal stenosis (2%) or shortened status (7%) had OPK. Six per cent of patients had neurological problems. However, 50% of the cohort from HSC had cognitive delays, and only cases from 2007 onwards reported cognitive delays (the prevalence was 17% among those cases). This review confirms the classical diagnostic features of BOS. In addition, it highlights a previously unreported association between a shortened stature and OPK, as well as a possible association with cognitive delays. [ABSTRACT FROM AUTHOR]

Many studies have aimed to identify the controllers of sweating using ventilated capsules with intradermal microdialysis. It is unclear, however, if the surface area covered by the capsule influences the observed response as a result of differences in the number of sweat glands affected by the infused pharmacological agent relative to the total glands captured by the capsule. We evaluated the area of skin perfused with agents delivered via microdialysis. Thereafter, we developed a specialized sweat capsule (1.1 cm2) and compared the sweating response with a classic capsule (2.8 cm2). In Protocol 1 ( n = 6), methacholine was delivered to forearm skin in a dose-dependent manner (1-2000 mmol L−1). The area of activated sweat glands was assessed via the modified iodine-paper technique. In Protocol 2 ( n = 6), the area of inhibited sweat glands induced by ouabain and atropine was assessed during moderate-intensity cycling. Marked variability in the affected skin area was observed (0.9 ± 0.4 to 5.2 ± 1.1 cm2). In Protocol 3 ( n = 6), we compared the attenuation in local sweat rate ( LSR) induced by atropine between the new and classic capsule during moderate-intensity cycling. Atropine attenuated sweating as assessed using the new (control: 0.87 ± 0.23 mg min−1 cm−2 vs. atropine: 0.54 ± 0.22 mg min−1 cm−2; P < 0.01) and classic (control: 0.85 ± 0.33 mg min−1 cm−2 vs. atropine: 0.60 ± 0.26 mg min−1 cm−2; P = 0.05) capsule designs. Importantly, responses did not differ between capsule designs ( P = 0.23). These findings provide critical information regarding the skin surface area perfused by microdialysis and suggest that use of a larger capsule does not alter the mechanistic insight into the sweating response gained when using microdialysis. [ABSTRACT FROM AUTHOR]

Hereditary sensory and autonomic neuropathy type III (HSAN III) features disturbed proprioception and a marked ataxic gait. We recently showed that joint angle matching error at the knee is positively correlated with the degree of ataxia. Using intraneural microelectrodes, we also documented that these patients lack functional muscle spindle afferents but have preserved large-diameter cutaneous afferents, suggesting that patients with better proprioception may be relying more on proprioceptive cues provided by tactile afferents. We tested the hypothesis that enhancing cutaneous sensory feedback by stretching the skin at the knee joint using unidirectional elasticity tape could improve proprioceptive accuracy in patients with a congenital absence of functional muscle spindles. Passive joint angle matching at the knee was used to assess proprioceptive accuracy in 25 patients with HSAN III and 9 age-matched control subjects, with and without taping. Angles of the reference and indicator knees were recorded with digital inclinometers and the absolute error, gradient, and correlation coefficient between the two sides calculated. Patients with HSAN III performed poorly on the joint angle matching test [mean matching error 8.0 ± 0.8° (±SE); controls 3.0 ± 0.3°]. Following application of tape bilaterally to the knee in an X-shaped pattern, proprioceptive performance improved significantly in the patients (mean error 5.4 ± 0.7°) but not in the controls (3.0 ± 0.2°). Across patients, but not controls, significant increases in gradient and correlation coefficient were also apparent following taping. We conclude that taping improves proprioception at the knee in HSAN III, presumably via enhanced sensory feedback from the skin. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of the present study was to develop and describe a simple method to evaluate the rate of ion reabsorption of eccrine sweat glands in human using the measurement of galvanic skin conductance (GSC) and local sweating rate (SR). This purpose was investigated by comparing the SR threshold for increasing GSC with following two criteria of sweat ion reabsorption in earlier studies such as (1) the SR threshold for increasing sweat ion was at approximately 0.2–0.5 mg/cm2/min and (2) exercise heat acclimation improved the sweat ion reabsorption ability and would increase the criteria 1.Methods: Seven healthy non-heat-acclimated male subjects received passive heat treatment both before and after 7 days of cycling in hot conditions (50% maximum oxygen uptake, 60 min/day, ambient temperature 32 °C, and 50% relative humidity).Results: Subjects became partially heat-acclimated, as evidenced by the decreased end-exercise heart rate (p < 0.01), rate of perceived exhaustion (p < 0.01), and oesophageal temperature (p = 0.07), without alterations in whole body sweat loss, from the first to the last day of training. As hypothesized, we confirmed that the SR threshold for increasing GSC was near the predicted SR during passive heating before exercise heat acclimation, and increased significantly after training (0.19 ± 0.09–0.32 ± 0.10 mg/cm2/min, p < 0.05).Conclusions: The reproducibility of sweat ion reabsorption by the eccrine glands in the present study suggests that the relationship between GSC and SR can serve as a new index for assessing the maximum rate of sweat ion reabsorption of eccrine sweat glands in humans. [ABSTRACT FROM AUTHOR]

Ophthalmic pathologic studies of retinoblastoma first definitively elucidated a genetic etiology for cancer three decades ago. Advances in DNA sequencing, protein expression profiling, and the exploration of epigenetics have since led to categorization of tumors and clinical prognostication based on genetic aberrancy. There are now many neoplasms that are defined by a characteristic genetic signature. In the past several years alone, much has been discovered in regard to the original tumor-suppressor gene initially defined in retinoblastoma as well as in other intraocular tumors such as medulloepithelioma. Our further understanding of ocular adnexal tumors that result in substantial morbidity and mortality, such as sebaceous carcinoma, has also benefited from a genetic approach. In this article, we review the clinicopathologic features of the foregoing three entities—retinoblastoma, medulloepithelioma, and sebaceous carcinoma—in order to highlight discoveries in their underlying abnormal molecular genetic functioning. [ABSTRACT FROM PUBLISHER]

Various cutaneous glands have been identified as sources of chemical signals that mediate many social interactions in deer. The pampas deer, a species considered near threatened, inhabits grasslands of South America. In this work, the chemical compositions from preorbital, tarsal, and digital gland secretions of semi-captive males were characterized by gas chromatography-mass spectrometry. The composition of these secretions showed a great complexity, with 143 compounds detected. Hierarchical cluster and principal component analyses show no relation to age or secretion type. Five compound classes (esters, fatty alcohols, lactones, sterols, and sulphuretted) differed with glands. The chemical complexity of the secretions, the individual differences in the whole compounds composition, and the absence of clustering by age lead to the hypothesis that these secretions may encode at once for individual information and for social status information. [ABSTRACT FROM AUTHOR]

Objective The purpose of this review was to determine the likelihood ofmalignancy or complications with ultrasonographic diagnosis of adnexal masses in pregnancy and to evaluate the obstetrical outcomes. Materials andMethods A review of literature was performed using keywords “adnexal mass and pregnancy” or “ovarian mass and pregnancy.” Results Out of the 340 abstracts reviewed, 313 were excluded. The incidence of adnexalmass in pregnancy varied from 0.1 to 2.4%, with an average of 0.02%. Regarding the likelihood of malignancy, in seven publications, there were 557 women with 563 adnexal masses. Of these 563 masses, 48% were classified as simple and 52% as complex. Among the simple masses, 1% were malignant. Among the complex masses, 9% were malignant. When comparing laparoscopy and laparotomy, the rate of preterm contractions was found to be higher in patients undergoing laparotomy and this was statistically significant. Other measures, such as spontaneous abortion, vaginal bleeding, < 37 week delivery, and intrauterine fetal demise, were not found to have a significant difference. Conclusion Adnexal masses in pregnancy occur infrequently and depending on whether the surgery was performed emergently or electively, via laparoscopy or laparotomy, the outcomes will vary. [ABSTRACT FROM AUTHOR]

Adolescence starts with the beginning of physiologically normal puberty, and ends when an adult identity and behavior are admitted. This period of development corresponds roughly to the period between the ages of 10 and 19 years, which is compatible the World Health Organization's definition of adolescence. Most of the adnexal masses during this period are functional ovarian cysts and benign neoplasms, and conservative surgery is suggested in the case of a cystic mass smaller than 8 cm. In all adolescents with adnexal masses, the preoperative evaluation should include a comprehensive medical record, physical examination, and imaging methods such as ultrasonography and laboratory examinations. The aim of this study was to evaluate preoperative findings, surgical procedure and histopathologic findings of the adolescent patients who had surgery for adnexal mass in our clinic. Totally 41 patients were included into study. Age, menstrual history, complaints during admission, images of the masses that were detected with different modalities, laboratory findings, applied surgical procedure and pathology findings were recorded. Among the symptoms that lead patients to admit hospital, abdominal pain was the first that was in 23 patients (56.1%). As we evaluate type of operation, 12 patients (29.3%) had laparoscopy, 29 patients (70.7%) had exploratory laparotomy operation. Mini laparotomy was the most often used incision type in 17 patients (41.5%). 34 patients (82.9%) had only cystectomy as an organ preserving surgery and most common histopathologic diagnosis was functional cyst in 14 patients (34.1%). In conclusion; since most pelvic masses in the adolescent period have benign character, surgery in this period should be minimally invasive and organ preserving for future fertility concerns and avoid from premature menopause [ABSTRACT FROM AUTHOR]