Cutaneous Ganglioneuromas With Overlying Epidermal Hyperplasia: A Case Series Presentation and Proposal of Potential Etiopathogenesis.

ABSTRACT: Cutaneous ganglioneuromas (GNs) are exceptionally uncommon tumors, and many reported cases describe association with overlying epidermal hyperplasia that may be interpreted as seborrheic keratosis (SK) or SK-like proliferation. We report 5 cases of cutaneous GN in adult patients; all of which were discovered incidentally in the immediate vicinity of epidermal hyperplasia. A review of the literature demonstrates the current-although likely imperfect-understanding of the etiopathogenesis of both SK and GN in the skin. We explore the putative pathophysiologies of other common, well-characterized skin lesions and, taking them into account, provide rationale for the coexistence of cutaneous GN with overlying SK and SK-like epidermal changes. However, we ultimately acknowledge a dilemma of causality and, given the rarity of their co-occurrence, objectively question whether occasional cameo appearances by GN lying subjacent to SK and SK-like hyperplasia may be due merely to chance.

There is fat in this sclerosis: A case report of sclerotic lipoma and review of the literature.

Sclerotic lipomas, a lipoma variant, are benign subcutaneous tumors, so-named because of their resemblance to sclerotic fibromas. Previous literature has suggested that these tumors may show a predilection for middle-aged adult males. We report an unusual case of a sclerotic lipoma diagnosed on the scalp of a 66-year-old female. The patient presented to the outpatient clinic with a 3- to 4-year history of an enlarging and irritated 2.6-cm nodule on the anterior crown of the scalp, clinically thought to be a pilar cyst. Histopathological examination from the excisional specimen revealed a well-circumscribed dermal to subcutaneous tumor with ample sclerotic collagen bundles, an increased number of CD34 positive spindled cells, and prominent S-100 positive mature adipocytes comprising greater than 50% of the tumor. We present this case given its atypical clinical and histopathological presentation, review the literature of sclerotic lipomas, and discuss the differential diagnosis to raise awareness of this rare entity.

Development of RET mutant cutaneous angiosarcoma during BRAF inhibitor therapy.

Treatment with BRAF inhibitors may lead to paradoxical mitogen-activated protein kinase (MAPK) pathway activation and accelerated tumorigenesis in cells with preexisting oncogenic hits. This phenomenon manifests clinically in the development of squamous cell carcinomas (SCCs) and keratoacanthomas (KAs) in patients treated with BRAF inhibitors. Cases of extracutaneous malignancies associated with BRAF inhibitors have also been reported. We present a case of a patient who developed a cutaneous angiosarcoma 6 months after initiation of vemurafenib therapy. Next-generation sequencing (NGS) revealed a mutation in RET, which lies upstream of the MAPK pathway. This case highlights that treatment with BRAF inhibitors may promote the accelerated growth of secondary malignancies. Physician awareness of the spectrum of secondary malignancies associated with BRAF inhibitor treatment will support their early detection and treatment.

Rapid detection of high-emitting vehicles by on-road remote sensing technology improves urban air quality.

Vehicle emissions are the most important source of air pollution in the urban environment worldwide, and their detection and control are critical for protecting public health. Here, we report the use of on-road remote sensing (RS) technology for fast, accurate, and cost-effective identification of high-emitting vehicles as an enforcement program for improving urban air quality. Using large emission datasets from chassis dynamometer testing, RS, and air quality monitoring, we found that significant percentages of in-use petrol and LPG vehicles failed the emission standards, particularly the high-mileage fleets. The RS enforcement program greatly cleaned these fleets, in terms of high-emitter percentages, fleet average emissions, roadside and ambient pollutant concentrations, and emission inventory. The challenges of the current enforcement program are conservative setting of cut points, single-lane measurement sites, and lack of application experience in diesel vehicles. Developing more accurate and vertical RS systems will improve and extend their applications.

Impact of drivers on real-driving fuel consumption and emissions performance.

Eco-driving has attracted great attention as a cost-effective and immediate measure to reduce fuel consumption significantly. Understanding the impact of driver behaviour on real driving emissions (RDE) is of great importance for developing effective eco-driving devices and training programs. Therefore, this study was conducted to investigate the performance of different drivers using a portable emission measurement system. In total, 30 drivers, including 15 novice and 15 experienced drivers, were recruited to drive the same diesel vehicle on the same route, to minimise the effect of uncontrollable real-world factors on the performance evaluation. The results show that novice drivers are less skilled or more aggressive than experienced drivers in using the accelerator pedal, leading to higher vehicle and engine speeds. As a result, fuel consumption rates of novice drivers vary in a slightly greater range than those of experienced drivers, with a marginally higher (2%) mean fuel consumption. Regarding pollutant emissions, CO and THC emissions of all drivers are well below the standard limits, while NOx and PM emissions of some drivers significantly exceed the limits. Compared with experienced drivers, novice drivers produce 17% and 29% higher mean NOx and PM emissions, respectively. Overall, the experimental results reject the hypothesis that driver experience has significant impacts on fuel consumption performance. The real differences lie in the individual drivers, as the worst performing drivers have significantly higher fuel consumption rates than other drivers, for both novice and experienced drivers. The findings suggest that adopting eco-driving skills could deliver significant reductions in fuel consumption and emissions simultaneously for the worst performing drivers, regardless of driving experience.

Simulation of engine faults and their impact on emissions and vehicle performance for a liquefied petroleum gas taxi.

The deterioration of emissions control systems in a spark ignition engine is predominantly a gradual process of wear and tear occurring as vehicles accumulate mileage. As new innovations in engine and emissions technology have been progressively introduced to meet lower emissions targets, the impact of gradual deterioration of hardware has become more challenging to identify and quantify in the repair industry. When a pioneering emissions control programme utilising remote sensing to detect high emitting gasoline and liquefied petroleum gas (LPG) vehicles was to be introduced in Hong Kong, it became apparent the repair industry needed specialised training to assist with identifying the types of failures which would lead to high vehicle emissions. To identify the impact of hardware deterioration and failures, a Toyota Crown Comfort LPG taxi was used to demonstrate simulated failures of engine hardware systems to measure their impact on emissions, fuel consumption and drivability using a chassis dynamometer. This novel study simulated a broad range of deterioration and failures covering the intake, fuel supply, ignition, and exhaust systems. The results of the study showed significant THC and CO increases of up to 317% (0.604 g/km) and 782% (5.351 g/km) respectively for a simulated oxygen sensor high voltage fault and a sticky mixture control valve. The largest increase in NOx emissions was for restricted main fuel supply in the LPG vapouriser, producing an increase of 282% (1.41 g/km). Fuel consumption varied with increases of up to 15.5%. Drivability was impacted with poor idle from a number of faults and especially by a worn throttlebody which produced rough acceleration characteristics as well. This study clearly highlights the importance of having properly maintained emissions and engine hardware systems to achieve optimal fuel economy and compliant emissions levels, which could be reproduced in other regions for prescribed emissions regulation.

Large eddy simulation of vehicle emissions dispersion: Implications for on-road remote sensing measurements.

On-road remote sensing technology measures the concentration ratios of pollutants over CO2 in the exhaust plume in half a second when a vehicle passes by a measurement site, providing a rapid, non-intrusive and economic tool for vehicle emissions monitoring and control. A key assumption in such measurement is that the emission ratios are constant for a given plume. However, there is a lack of study on this assumption, whose validity could be affected by a number of factors, especially the engine operating conditions and turbulence. To guide the development of the next-generation remote sensing system, this study is conducted to investigate the effects of various factors on the emissions dispersion process in the vehicle near-wake region and their effects on remote sensing measurement. The emissions dispersion process is modelled using Large Eddy Simulation (LES). The studied factors include the height of the remote sensing beam, vehicle speed, acceleration and side wind. The results show that the measurable CO2 and NO exhaust plumes are relatively short at 30 km/h cruising speed, indicating that a large percentage of remote sensing readings within the measurement duration (0.5 s) are below the sensor detection limit which would distort the derived emission ratio. In addition, the valid measurement region of NO/CO2 emission ratio is even shorter than the measurable plume and is at the tailpipe height. The effect of vehicle speed (30-90 km/h) on the measurable plume length is insignificant. Under deceleration condition, the length of the valid NO/CO2 measurement region is shorter than under cruising and acceleration conditions. Side winds from the far-tailpipe direction have a significant effect on remote sensing measurements. The implications of these findings are discussed and possible solutions to improve the accuracy of remote sensing measurement are proposed.

Statistical evaluation of on-road vehicle emissions measurement using a dual remote sensing technique.

On-road remote sensing (RS) is a rapid, non-intrusive and economical tool to monitor and control the emissions of in-use vehicles, and currently is gaining popularity globally. However, a majority of studies used a single RS technique, which may bias the measurements since RS only captures a snapshot of vehicle emissions. This study aimed to use a unique dual RS technique to assess the characteristics of on-road vehicle emissions. The results show that instantaneous vehicle emissions are highly dynamic under real-world driving conditions. The two emission factors measured by the dual RS technique show little correlation, even under the same driving condition. This indicates that using the single RS technique may be insufficient to accurately represent the emission level of a vehicle based on one measurement. To increase the accuracy of identifying high-emitting vehicles, using the dual RS technique is essential. Despite little correlation, the dual RS technique measures the same average emission factors as the single RS technique does when a large number of measurements are available. Statistical analysis shows that both RS systems demonstrate the same Gamma distribution with ≥200 measurements, leading to converged mean emission factors for a given vehicle group. These findings point to the need for a minimum sample size of 200 RS measurements in order to generate reliable emission factors for on-road vehicles. In summary, this study suggests that using the single or dual RS technique will depend on the purpose of applications. Both techniques have the same accuracy in calculating average emission factors when sufficient measurements are available, while the dual RS technique is more accurate in identifying high-emitters based on one measurement only.

Evaluating in-use vehicle emissions using air quality monitoring stations and on-road remote sensing systems.

This study investigated real world in-use vehicle emissions using two regulatory techniques simultaneously, namely on-road remote sensing (RS) systems and air quality (AQ) monitoring stations, aiming to provide a full pollution profile from tailpipe to roadside and atmosphere. Two large AQ and RS datasets collected during 2012-2018 were analyzed. The effects of various emission control programmes on the trends of tailpipe emissions and air quality were evaluated. Correlations between tailpipe emissions and roadside and ambient air quality were also explored. The results showed a decreasing trend of NO2 at both roadside and ambient AQ stations from 2013 to 2016, which was attributed to the intensive implementation of a series of vehicle emissions control programmes. Although NO2 was decreasing, O3 was generally increasing for all AQ stations. AQ data showed that O3 had little correlation with either NO2 or NOx, but was mainly determined by NO2/NOx ratio. Roadside NO2/NOx ratio increased first and then decreased or stabilized after 2014, while ambient NO2/NOx ratio increased steadily. RS data showed that the overall NO decreased quickly during 2012-2015 and then decreased moderately after 2015. The decrease was mainly attributed to the effective NO reduction from LPG vehicles. However, diesel NO remained high and reduced relatively slowly during the study period. Gasoline vehicles were relatively clean compared with LPG and diesel vehicles. Finally, good correlations were demonstrated between NO measured by RS sites and NOx measured by roadside AQ stations, indicating that vehicle emissions were the major contributor to roadside NOx pollution. Ambient NOx emissions could be affected by various sources, leading to different correlation levels between RS and ambient AQ results.

Leukemia cutis (involving chronic lymphocytic leukemia) within excisional specimens: a series of 6 cases.

We present 6 cases of chronic lymphocytic leukemia (CLL) that incidentally involved 6 excisional specimens for biopsy-proven carcinoma. CLL was notably absent from all 5 biopsies that were available for review. In 2 of 6 cases, this was the patients' initial presentation of CLL. Five of 6 cases involved routine paraffin-embedded tissue specimens and 1 case involved frozen tissue sections from a Mohs surgical procedure. The mean age range of the patients was 84 years. Only one of 5 patients in which we have follow-up data, died of a CLL-related cause at the time of this submission (mean follow-up 19.8 months). On histologic examination, the most common pattern of involvement by CLL (as seen in 4 of the 6 cases) was a dense, nodular, and superficial and deep perivascular, periadnexal, and perineural infiltrate beneath the fibrosing granulation tissue of the prior biopsy site. The infiltrate involved the upper and deep reticular dermis and subcutaneous fat. The remaining 2 cases demonstrated a novel finding of a subtle infiltration of leukemic cells among extravasated red blood cells within the mid and deep reticular dermis. In all cases, leukemic cells were present as tightly packed, small, monomorphous, hyperchromatic lymphocytes and 1 case demonstrated a proliferation center. Immunohistochemical stains were performed on 3 of 6 cases, and the leukemic cells were CD5/CD20/CD23/CD3. This case series raises awareness that CLL can incidentally involve dermatopathology specimens and occasionally be the initial presentation of the patients' systemic illness. This series also highlights the unique histologic patterns of CLL in the skin, one of which has not been previously described, and illustrates how these patterns are distinct from the typical interstitial infiltration seen in other cases of leukemia cutis.

Fuel consumption and emissions performance under real driving: Comparison between hybrid and conventional vehicles.

Hybrid electric vehicles (HEVs) are perceived to be more energy efficient and less polluting than conventional internal combustion engine (ICE) vehicles. However, increasing evidence has shown that real-driving emissions (RDE) could be much higher than laboratory type approval limits and the advantages of HEVs over their conventional ICE counterparts under real-driving conditions have not been studied extensively. Therefore, this study was conducted to evaluate the real-driving fuel consumption and pollutant emissions performance of HEVs against their conventional ICE counterparts. Two pairs of hybrid and conventional gasoline vehicles of the same model were tested simultaneously in a novel convoy mode using two portable emission measurement systems (PEMSs), thus eliminating the effect of vehicle configurations, driving behaviour, road conditions and ambient environment on the performance comparison. The results showed that although real-driving fuel consumption for both hybrid and conventional vehicles were 44%-100% and 30%-82% higher than their laboratory results respectively, HEVs saved 23%-49% fuel relative to their conventional ICE counterparts. Pollutant emissions of all the tested vehicles were lower than the regulation limits. However, HEVs showed no reduction in HC emissions and consistently higher CO emissions compared to the conventional ICE vehicles. This could be caused by the frequent stops and restarts of the HEV engines, as well as the lowered exhaust gas temperature and reduced effectiveness of the oxidation catalyst. The findings therefore show that while achieving the fuel reduction target, hybridisation did not bring the expected benefits to urban air quality.

Characterisation of diesel vehicle emissions and determination of remote sensing cutpoints for diesel high-emitters.

Diesel vehicles are a major source of air pollutants in cities and have caused significant health risks to the public globally. This study used both on-road remote sensing and transient chassis dynamometer to characterise emissions of diesel light goods vehicles. A large sample size of 183 diesel vans were tested on a transient chassis dynamometer to evaluate the emission levels of in-service diesel vehicles and to determine a set of remote sensing cutpoints for diesel high-emitters. The results showed that 79% and 19% of the Euro 4 and Euro 5 diesel vehicles failed the transient cycle test, respectively. Most of the high-emitters failed the NO limits, while no vehicle failed the HC limits and only a few vehicles failed the CO limits. Vehicles that failed NO limits occurred in both old and new vehicles. NO/CO2 ratios of 57.30 and 22.85 ppm/% were chosen as the remote sensing cutpoints for Euro 4 and Euro 5 high-emitters, respectively. The cutpoints could capture a Euro 4 and Euro 5 high-emitter at a probability of 27% and 57% with one snapshot remote sensing measurement, while only producing 1% of false high-emitter detections. The probability of high-emitting events was generally evenly distributed over the test cycle, indicating that no particular driving condition produced a higher probability of high-emitting events. Analysis on the effect of cutpoints on real-driving diesel fleet was carried out using a three-year remote sensing program. Results showed that 36% of Euro 4 and 47% of Euro 5 remote sensing measurements would be detected as high-emitting using the proposed cutpoints.

A Sweet Diagnosis for a Non-Resolving Rash.

Subcutaneous Sweet's syndrome (SSS) is a rare variant of Sweet's syndrome (SS), clinically characterized by erythematous plaques or nodules with a histologic pattern demonstrating a neutrophilic panniculitis (NP). We report a case of a 74-year-old woman with myelodysplastic syndrome (MDS) who presented with persistent fever, malaise, and non-resolving generalized erythematous nodules and was found to have an MDS-related SSS. SSS should be entertained and other causes of NP should be excluded prior to treating a patient with systemic corticosteroids. Early diagnosis of SSS in a patient not responding to broad-spectrum antibiotics is crucial as it helps to minimize unnecessary prolonged antibiotics exposure in this era of antimicrobial resistance. In patients with frequent relapses, a slow corticosteroid taper could be beneficial.

Emission measurement of diesel vehicles in Hong Kong through on-road remote sensing: Performance review and identification of high-emitters.

A two-year remote sensing measurement program was carried out in Hong Kong to obtain a large dataset of on-road diesel vehicle emissions. Analysis was performed to evaluate the effect of vehicle manufacture year (1949-2015) and engine size (0.4-20 L) on the emission rates and high-emitters. The results showed that CO emission rates of larger engine size vehicles were higher than those of small vehicles during the study period, while HC and NO were higher before manufacture year 2006 and then became similar levels between manufacture years 2006 and 2015. CO, HC and NO of all vehicles showed an unexpectedly increasing trend during 1998-2004, in particular ≥6001 cc vehicles. However, they all decreased steadily in the last decade (2005-2015), except for NO of ≥6001 cc vehicles during 2013-2015. The distributions of CO and HC emission rates were highly skewed as the dirtiest 10% vehicles emitted much higher emissions than all the other vehicles. Moreover, this skewness became more significant for larger engine size or newer vehicles. The results indicated that remote sensing technology would be very effective to screen the CO and HC high-emitters and thus control the on-road vehicle emissions, but less effective for controlling NO emissions. No clear correlation was observed between the manufacture year and percentage of high-emitters for ≤3000 cc vehicles. However, the percentage of high-emitters decreased with newer manufacture year for larger vehicles. In addition, high-emitters of different pollutants were relatively independent, in particular NO emissions, indicating that high-emitter screening criteria should be defined on a CO-or-HC-or-NO basis, rather than a CO-and-HC-and-NO basis.

Tackling nitric oxide emissions from dominant diesel vehicle models using on-road remote sensing technology.

Remote sensing provides a rapid detection of vehicle emissions under real driving condition. Remote sensing studies showed that diesel nitrogen oxides emissions changed little or were even increasing in recent years despite the tightened emission standards. To more accurately and fairly evaluate the emission trends, it is hypothesized that analysis should be detailed for individual vehicle models as each model adopted different emissions control technologies and retrofitted the engine/vehicle at different time. Therefore, this study was aimed to investigate the recent nitric oxide (NO) emission trends of the dominant diesel vehicle models using a large remote sensing dataset collected in Hong Kong. The results showed that the diesel vehicle fleet was dominated by only seven models, accounting for 78% of the total remote sensing records. Although each model had different emission levels and trends, generally all the dominant models showed a steady decrease or stable level in the fuel based NO emission factors (g/kg fuel) over the period studied except for BaM1 and BdM2. A significant increase was observed for the BaM1 2.49 L and early 2.98 L models during 2005-2011, which we attribute to the change in the diesel fuel injection technology. However, the overall mean NO emission factor of all the vehicles was stable during 1991-2006 and then decreased steadily during 2006-2016, in which the emission trends of individual models were averaged out and thus masked. Nevertheless, the latest small, medium and heavy diesel vehicles achieved similar NO emission factors due to the converging of operation windows of the engine and emission control devices. The findings suggested that the increasingly stringent European emission standards were not very effective in reducing the NO emissions of some diesel vehicle models in the real world.

Multicentric reticulohistiocytosis presenting with clinical features of dermatomyositis.

Multicentric reticulohistiocytosis (MRH) is a rare disorder with proliferating histiocytes that develop into multinucleated giant cells with "ground-glass" cytoplasm. The disease presents with a rapidly destructive, sometimes permanently debilitating, polyarthritis and a papulonodular eruption, generally of the face and hands. We present 3 cases of MRH in which the initial clinical diagnosis was thought to be dermatomyositis (DM). The cutaneous findings in these cases included an erythematous, predominantly photodistributed macular and papular eruption clinically consistent with DM. However, skin biopsy specimens revealed the diagnosis of MRH. This previously unreported clinical similarity between MRH and DM is significant because the treatment options and future complications vary greatly between the 2 diseases. Thus, it is important to consider the possibility of multicentric reticulohistiocytosis when evaluating a patient with the clinical diagnosis of dermatomyositis.