Morphoea presenting histopathologically as mycosis fungoides: an illustrative series of four cases.

AIMS: There have been exceptional reports of morphoea presenting with epidermal changes overlapping histopathologically with cutaneous T cell lymphoma of the mycosis fungoides type (MF). This phenomenon gives rise to an ambiguous clinicopathological scenario in which distinguishing these conditions may be challenging. The aim of this study is to characterise the clinical, histopathological and molecular findings of this phenomenon through a case series. METHODS AND RESULTS: Four patients with classical clinical presentation of morphoea but unusual histopathology displaying typical findings of morphoea, together with intra-epidermal CD8 positive lymphocytes indistinguishable from MF, were identified. The clinical phenotypes of morphoea were varied, and they all presented early in the active phase of the disease. They all exhibited intra-epidermal lymphocytes with tagging and cytological atypia. Pautrier-like microabscesses were also seen. Using molecular analysis, two cases showed clonal TCR gene rearrangement. Follow-up of all cases has been consistent with classical morphoea. CONCLUSION: Early morphoea can seldom present with atypical clonal intra-epidermal lymphocytes indistinguishable from MF. The fact that these changes can occur in several different clinical subtypes of morphoea raises the possibility that this could be a pattern of inflammation in early disease more common than currently appreciated.

How to critically appraise a systematic review: an aide for the reader and reviewer.

The number of published systematic reviews has soared rapidly in recent years. Sadly, the quality of most systematic reviews in dermatology is substandard. With the continued increase in exposure to systematic reviews, and their potential to influence clinical practice, we sought to describe a sequence of useful tips for the busy clinician reader to determine study quality and clinical utility. Important factors to consider when assessing systematic reviews include: determining the motivation to performing the study, establishing if the study protocol was prepublished, assessing quality of reporting using the PRISMA checklist, assessing study quality using the AMSTAR 2 critical appraisal checklist, assessing for evidence of spin, and summarizing the main strengths and limitations of the study to determine if it could change clinical practice. Having a set of heuristics to consider when reading systematic reviews serves to save time, enabling assessment of quality in a structured way, and come to a prompt conclusion of the merits of a review article in order to inform the care of dermatology patients.

Delusional infestation in healthcare professionals: Outcomes from a multi-centre case series.

Delusional infestation (DI) describes an unwavering fixed belief of infestation with pathogens, despite a lack of medical evidence supporting this. Effective management of DI with antipsychotics is made challenging by the fixed belief that the condition is an infestation or infection rather than a mental illness. A case series of individuals diagnosed with DI included 11% who were healthcare professionals (HCPs). We sought to characterise a cohort of HCPs who presented with DI in the UK. The case notes of HCPs diagnosed with DI at specialist clinics between 2015 and 2019 were reviewed. Demographic and clinical data were obtained. Twelve HCPs were identified out of a total of 381 individuals diagnosed with DI. Median age was 52.5 (IQR = 14.5) years. 75% (n = 9) were women. Ten individuals had primary DI, whilst two had secondary DI (one to recreational drug use, one to depression). Four individuals (33%) engaged with antipsychotic treatment. Two responded well, both had secondary DI. Of the two individuals with primary DI who engaged, one did not respond to antipsychotic medication and the other was unable to tolerate two antipsychotic drugs. In Primary DI (n = 10), the rate of adherence was lower at 20% (n = 2). In DI, high engagement and adherence rates to treatment have been reported in specialist centres. Improvement has been reported as high as 70%-75%. This indicates that a large proportion of individuals who adhere to treatment appear to derive benefit. In this series, engagement with treatment by HCPs with primary DI was low at 20%, and improvement was only achieved in individuals with secondary DI. Mental illness-related stigma, feelings of distress and difficulty forming therapeutic relationships with a professional peer are significant challenges. Developing rapport is key to treatment success in DI. In HCPs this may be suboptimal due to these negative feelings, resulting in lower engagement. A diagnosis of DI in a HCP may raise concerns regarding fitness to practise. An assessment of the impact of DI and the potential to interfere with professional duties warrants consideration. We highlight the occurrence of DI in HCPs, and the apparent lower engagement with treatment in this cohort.

Tuberous xanthoma secondary to homozygous familial hypercholesterolaemia: a life-threatening diagnosis.

We report the case of a 9-year-old girl who presented with asymptomatic lesions on the extensor surfaces of the elbows and knees, in keeping with tuberous xanthoma. She was investigated and diagnosed with homozygous familial hypercholesterolaemia, and commenced on lipid-lowering treatment. We highlight the importance of identification of this condition early, such that life-saving treatment can be initiated and premature death avoided. Click here for the corresponding questions to this CME article.

Eosinophilic infiltration of oesophagus following carbamazepine-induced drug reaction with eosinophilia and systemic symptoms: an under-reported sequel?

We report a patient with carbamazepine-induced drug reaction with eosinophilia and systemic symptoms (DRESS), who developed painful dysphagia in the follow-up period. Gastrointestinal, including oesophageal, complications are rarely reported following DRESS, and we wish to highlight this possibly under-reported phenomenon.

Social Disadvantage and Social Isolation Are Associated With a Higher Resting Heart Rate: Evidence From The Irish Longitudinal Study on Ageing.

OBJECTIVES: A high resting heart rate (RHR) represents a major risk factor for cardiovascular disease and individuals from poorer backgrounds have a higher RHR compared with their more advantaged peers. This study investigates the pathways through which low socioeconomic status (SES) contributes to a higher RHR. METHOD: The sample involved data for 4,888 respondents who were participating in the first wave of The Irish Longitudinal Study on Ageing. Respondents completed a detailed interview at home and underwent a 5-min baseline electrocardiograph recording as part of a clinic-based health assessment. SES was indexed using household income. RESULTS: The mean difference in RHR between those at polarized ends of the income distribution was 2.80 beats per minute (bpm) (95% CI = 1.54, 4.06; p < .001), with the magnitude of the socioeconomic differential being greater for men (4.15 bpm; 95% CI = 2.18, 6.12; p < .001) compared with women (1.57 bpm; 95% CI = 0.04, 3.10; p < .05). Psychosocial factors including social network size and loneliness accounted for a sizeable proportion of the socioeconomic differential in RHR, particularly among men. DISCUSSION: The finding that poorer people have a higher RHR reinforces the need for additional research exploring the pathways through which social inequalities are translated into biological inequalities.

AF is associated with self-reported syncope and falls in a general population cohort.

BACKGROUND: syncope is an important, but underestimated clinical problem in older persons. It is often overlooked in clinical practice or mistaken for falls. Atrial fibrillation (AF) is the most common cardiac arrhythmia, but little evidence exists regarding the association between AF, falls and syncope in the general population. METHODS: cross-sectional analyses within a population sample of people aged 50+, taken from The Irish Longitudinal Study on Ageing. Ten-minute electrocardiogram recordings (n = 4,885) were analysed to detect AF. Syncope (self-reported faints or blackouts) and falls in the past year, co-morbidities, health measures and medications were gathered through computer-aided personal interviews. Multivariable logistic regression was performed to study associations between AF, falls and syncope. RESULTS: mean age was 62 years (range: 50-91), 54% were female. Prevalence of AF was 3%, increasing to 8% in participants aged 75+. Of participants, 5% (n = 223) reported syncope and 20% (n = 972) reported falls. After adjustment for confounders, AF was significantly associated with faints and blackouts (odds ratio (OR) 2.0 [95% confidence interval (CI) 1.0-3.9]). After stratification by age category, we found that this association was strongest and only significant in participants aged 50-64 years (OR 4.4 [1.5-12.6]). Stratified for age group, AF was significantly associated with falls in participants aged 65-74 years (OR 2.0 [1.0-4.1]). CONCLUSIONS: adults aged 50+ with self-reported syncope and adults aged 65-74 years with falls are twice as likely to have AF at physical examination. These associations are independent of stroke, cardiovascular and psychotropic drugs and other confounders. Further longitudinal studies are needed to explore this association and potential causality further.

Recent syncope and unexplained falls are associated with poor cognitive performance.

OBJECTIVE: To compare cognitive performance in participants with and without syncope and unexplained falls in a large population representative sample aged 50 years or older. METHODS: Participants of the Irish longitudinal study on ageing (TILDA) were studied. Participants with a history of syncope and/or unexplained falls in the past 12 months were compared with those with no reported events. Cognitive performance was measured using the Montreal cognitive assessment (MoCA) score. Multivariate linear regression analysis controlling for potential confounders was performed to compare cognitive function by syncope and falls status. RESULTS: Five thousand eight hundred and forty-six participants were analysed, median age 62 years (inter-quartile range=14), and 54% were female. Five hundred and forty-nine (9.4%) had a syncopal event and/or an unexplained fall in past 12 months. One hundred and two (1.8%) subjects had two-plus syncopal events in the same period. There was a significant association between syncope/falls history and lower MoCA score, following adjustment for all confounders (B=-0.4; -0.69, -0.11; P=0.006). Higher syncope burden was also associated with lower performance; however, this was largely explained by confounders. There was no age interaction with these findings. CONCLUSION: Participants who experienced syncope and/or non-accidental falls in the previous year have poor global cognitive performance compared with case-controls. There was no effect of age on our results. Further investigation of the association between syncope burden, unexplained falls and cognitive decline is required to establish a relationship between these disorders.

Cognitive performance in orthostatic hypotension: findings from a nationally representative sample.

OBJECTIVES: To compare the cognitive profile of a population representative sample with orthostatic hypotension (OH) with the profile of a sample without. DESIGN: Cross-sectional analysis of a prospective nationally representative population study. SETTING: The Irish Longitudinal Study on Ageing (TILDA). PARTICIPANTS: TILDA participants (N = 5,936; mean age 63 ± 9, 54% female). MEASUREMENTS: OH was defined as a drop of 20 mmHg or more in systolic blood pressure or of 10 mmHg or more in diastolic blood pressure on standing from a seated position. Cognitive performance was assessed using comprehensive cognitive tests measuring domains of global function, executive function, processing speed, attention, and memory, from which composite standardized scores were computed. Multivariate analysis controlling for potential confounders was performed to compare cognitive performance according to OH status. RESULTS: Prevalence of OH was 6.1% (95% confidence interval = 5.4-6.7%). A significant negative association between OH status and global cognitive function (b = 0.21, P = .01) and memory (b = 0.26, P = .002) was found in women aged 65 and older after adjustment for demographic characteristics, mental health, cardiovascular disease, and medications (antihypertensive and antipsychotic), but other specific cognitive domains were not affected. CONCLUSION: OH was associated with poorer global cognitive function and poorer memory, independent of potential confounders, in women in a large population-based sample of older adults. Longitudinal studies with concomitant assessment of cerebral perfusion are needed to determine causal relationships.

Orthostatic hypotension is associated with lower cognitive performance in adults aged 50 plus with supine hypertension.

OBJECTIVES: This study investigated the association between orthostatic hypotension (OH), supine hypertension (SH), and cognitive performance. METHODS: Four thousand six hundred and ninety participants of The Irish Longitudinal Study on Ageing (TILDA) were studied. SH was defined as systolic blood pressure (SBP) greater than or equal to 140 mmHg and/or diastolic blood pressure (DBP) greater than or equal to 90 mmHg, measured following supine rest (10 minutes). OH was defined as a sustained drop of greater than or equal to 20 mmHg SBP or greater than or equal to 10 mmHg DBP at 20, 30, 60, and 90 seconds following orthostasis. Cognitive performance tests assessed global function, executive function, processing speed, memory, and attention from which z-scores were computed. Multivariate adjusted analysis was performed comparing cognitive scores by OH status overall and in SH and non-SH groups separately. RESULTS: Thirty-nine percent had baseline SH (n = 1,868) and demonstrated a greater orthostatic fall in SBP (p < .0001) and DBP (p < .0001). This group had a higher prevalence of OH at all time-points, and scored lower in tests across all cognitive domains. No overall association between OH and cognitive performance was seen. However, SH subjects with OH scored significantly worse (adjusted) than SH subjects without OH, in domains of global cognition (30 seconds poststand ß = -0.15; 99% confidence interval -0.29, -0.14; p = .004) and executive function (20 seconds poststand; ß = -0.11; 99% confidence interval -0.22, -0.01; p = .006). There was also an indication toward lower cognition in all nonsignificant analyses. OH was not associated with cognitive performance in non-SH subjects. CONCLUSION: In conclusion, individuals with SH (defined as BP > 140/90 mmHg) coupled with OH measured using phasic BP had lower global and executive cognitive performance than those with SH but without OH.

Cognitive function is associated with impaired heart rate variability in ageing adults: the Irish longitudinal study on ageing wave one results.

OBJECTIVE: To examine the independent association between heart rate variability (HRV) and cognitive performance, in a nationally representative population study of older adults. METHODS: Cross-sectional analysis of wave 1 data from the Irish longitudinal study on ageing (TILDA) was performed. A subset of 4,763 participants who underwent ECG recording during resting and paced breathing periods were used for the analysis. HRV indices were divided into quintiles for comparison of values and cognitive performance was defined using the Montreal cognitive assessment (MOCA) score. Multivariate linear regression was used to model the association between cognition and different quintiles of each HRV index, after adjustment for covariates. RESULTS: The mean age was 61.7 ± 8.3 years and 2,618 (55 %) were female. Lower quintiles of SDNN (P = 0.01-paced), LF (P = 0.001-paced), and LF:HF ratio (P = 0.049-paced) were significantly associated with lower MOCA scores (during both recording periods), independent of confounders. Sub-domains of MOCA responsible for the relationship were predominantly memory recall and language. INTERPRETATION: Reduced HRV is significantly associated with lower cognitive performance at a population level in people aged 50 and older. This further strengthens the relationship between autonomic dysfunction and cognitive disorders.

Normative values of cognitive and physical function in older adults: findings from the Irish Longitudinal Study on Ageing.

OBJECTIVES: To provide normative values of tests of cognitive and physical function based on a large sample representative of the population of Ireland aged 50 and older. DESIGN: Data were used from the first wave of The Irish Longitudinal Study on Ageing (TILDA), a prospective cohort study that includes a comprehensive health assessment. SETTING: Health assessment was undertaken at one of two dedicated health assessment centers or in the study participant's home if travel was not practicable. PARTICIPANTS: Five thousand eight hundred ninety-seven members of a nationally representative sample of the community-living population of Ireland aged 50 and older. Those with severe cognitive impairment, dementia, or Parkinson's disease were excluded. MEASUREMENTS: Measurements included height and weight, normal walking speed, Timed Up-and-Go, handgrip strength, Mini-Mental State Examination (MMSE), Montreal Cognitive Assessment (MoCA), Color Trails Test, and bone mineral density. Normative values were estimated using generalized additive models for location shape and scale (GAMLSS) and are presented as percentiles, means, and standard deviations. RESULTS: Generalized additive models for location shape and scale fit the observed data well for each measure, leading to reliable estimates of normative values. Performance on all tasks decreased with age. Educational attainment was a strong determinant of performance on all cognitive tests. Tests of walking speed were dependent on height. Distribution of body mass index did not change with age, owing to simultaneous declines in weight and height. CONCLUSION: Normative values were found for tests of many aspects of cognitive and physical function based on a representative sample of the general older Irish population.