Septin-5 and -7-IgGs: Neurologic, Serologic, and Pathophysiologic Characteristics.

BACKGROUND AND OBJECTIVES: We sought to determine clinical significance of neuronal septin autoimmunity and evaluate for potential IgG effects. METHODS: Septin-IgGs were detected by indirect immunofluorescence assays (IFAs; mouse tissue and cell based) or Western blot. IgG binding to (and internalization of) extracellular septin epitopes were evaluated for by live rat hippocampal neuron assay. The impact of purified patient IgGs on murine cortical neuron function was determined by recording extracellular field potentials in a multielectrode array platform. RESULTS: Septin-IgGs were identified in 23 patients. All 8 patients with septin-5-IgG detected had cerebellar ataxia, and 7 had prominent eye movement disorders. One of 2 patients with co-existing septin-7-IgG had additional psychiatric phenotype (apathy, emotional blunting, and poor insight). Fifteen patients had septin-7 autoimmunity, without septin-5-IgG detected. Disorders included encephalopathy (11; 2 patients with accompanying myelopathy, and 2 were relapsing), myelopathy (3), and episodic ataxia (1). Psychiatric symptoms (≥1 of agitation, apathy, catatonia, disorganized thinking, and paranoia) were prominent in 6 of 11 patients with encephalopathic symptoms. Eight of 10 patients with data available (from 23 total) improved after immunotherapy, and a further 2 patients improved spontaneously. Staining of plasma membranes of live hippocampal neurons produced by patient IgGs (subclasses 1 and 2) colocalized with pre- and post-synaptic markers. Decreased spiking and bursting behavior in mixed cultures of murine glutamatergic and GABAergic cortical neurons produced by patient IgGs were attributable to neither antigenic crosslinking and internalization nor complement activation. INTERPRETATION: Septin-IgGs are predictive of distinct treatment-responsive autoimmune central nervous system (CNS) disorders. Live neuron binding and induced electrophysiologic effects by patient IgGs may support septin-specific pathophysiology. ANN NEUROL 2022;92:1090-1101.

Malignancy-associated generalised exfoliative dermatitis: A retrospective study in a single-centre Asian cohort.

BACKGROUND: Erythroderma is an inflammatory skin condition that causes extensive erythema and skin scaling amounting ≥90% of the body surface area. This retrospective cohort study describes the prevalence of malignancy-associated erythroderma in a single centre where there was concerted effort to systematically offer malignancy screens to all adult erythroderma patients above the age of 65 years. METHODS: Clinical charts were reviewed for all adult inpatients and outpatients with erythroderma who attended the National University Hospital (NUH) from 1 July 2019 to 31 December 2021. Data collected included patient demographics, clinical findings, laboratory investigations, disease-specific investigations such as endoscopic procedures and biopsies, follow-up duration and mortality data. RESULTS: Seventy-four patients were analysed. The median age of the patients was 73 years old (interquartile range: 59-81 years old). An underlying dermatosis was the most common cause of erythroderma-63 patients having atopic dermatitis/asteatotic eczema or psoriasis. Three patients had erythroderma from drug eruptions, and 1 patient had chronic actinic dermatitis. Four patients had associated malignancies (5.4%). Half of our patients completed further evaluation for malignancy (52.7%). The rest had either declined or were eventually unable to complete the investigations. There was a higher prevalence of associated malignancy (7.8%) in elderly patients above 65 years old. CONCLUSION: When compared to existing literature, our cohort reflects a higher observed occurrence of malignancy in association with erythroderma. As delays in evaluation for underlying malignancy could result in potentially deleterious outcomes, it is prudent to consider systematic screening for malignancy in high-risk populations such as elderly erythroderma patients.

Hidradenitis suppurativa in pediatric patients.

Interest in and research on hidradenitis suppurativa (HS) have increased during the past decade, resulting in expanded knowledge about associated comorbidities and treatment efficacy. Knowledge about pediatric-onset HS is still limited, based on case studies, small case series, and extrapolation from adult studies. With increasing prevalence of childhood obesity, decreasing pubertal age, and increasing awareness of HS, physicians may start encountering younger HS patients. This review presents an updated discussion on the epidemiology, pathophysiology, and associated comorbidities in HS, with a focus on pediatric and adolescent patients. It also suggests recommendations for investigation and treatment based on current evidence.

Supportive oncodermatology-a narrative review of its utility and the way forward.

Supportive oncodermatology is an interdisciplinary field, emerging due to increasing dermatological morbidity in patients with cancer and the recognition of the need for greater collaborative and integrated care to improve patient outcomes. These two unique fields (Oncology and Dermatology) may be integrated in various ways, such as through specialised combined clinics, protocols for expedited access, multidisciplinary groups and meetings, and the development of best practices guidelines. This narrative review consolidates the small but growing literature surrounding supportive oncodermatology; discusses the potential benefit and disadvantages, and areas for future research; and suggests a framework for implementation.

Efficacy of targeted education in reducing topical steroid phobia: A randomized clinical trial.

BACKGROUND: Fear of adverse effects of corticosteroids is common in dermatology and results in medication nonadherence. OBJECTIVE: To study the efficacy of targeted education in reducing topical steroid phobia. METHODS: In this double-blinded, randomized controlled trial, participants in the intervention arm were presented with an educational video and patient information leaflet targeting common misconceptions of topical corticosteroids. Steroid phobia was assessed with the topical corticosteroid phobia (TOPICOP) scale, medication adherence with the Elaboration d'un outil d'evaluation de l'observance des traitements medicamenteux (ECOB) score, and quality of life with the Dermatology Life Quality Index (DLQI). RESULTS: The study randomized 275 patients. The mean TOPICOP score in the intervention arm decreased (improved) from 41.9 (SD, 17.4) to 37.1 (SD, 20.0) and to 33.8 (SD, 19.0) at 1 month and 3 months, respectively, with the reduction arising from the knowledge domain but not the fears and behaviors domain. This remained statistically significant after adjusting for demographic confounding with an expected reduction of 4.22 points (P = .031). After accounting for demographic factors, there was no statistical difference in medication adherence and quality of life. Limitations include the exclusion of non-English-speaking patients. CONCLUSION: Targeted education at a single time point improved the TOPICOP score primarily in the knowledge domain but not in the fear domain.

Radiofrequency ablation-induced thermal cutaneous injury in a neonate: A potential complication after intrauterine radiofrequency ablation.

Over the years, the application and complications of radiofrequency ablation (RFA) in selective fetal reduction for complex pregnancies have been increasingly documented. Despite its rising use in the field of obstetrics and gynecology, the cutaneous complications of RFA have not been commonly reported. Here, we present a case of cutaneous thermal injury to the fetus likely secondary to intrauterine RFA for fetal reduction.

Basaloid follicular hamartoma: clinical, dermoscopic, and histopathological characteristics of case.

Basaloid follicular hamartoma (BFH) is a rare benign adnexal tumor with variable clinical presentation. We report a case of a 64-year-old man, who presented with an incidental finding of a 3mm hyperpigmented macule on his cheek. Dermoscopy revealed a structureless blue lesion. Histopathology examination showed interconnecting lobules and cords of bland pigmented epithelial cells within the dermal stroma, with the presence of pseudohorncysts. The lesional cells were faintly positive for Bcl2on immunohistochemical staining. These findings were consistent with basaloid follicular hamartoma. Histological differential diagnoses include benign lesions such as trichoepithelioma, and malignant lesions such as basal cell carcinoma (BCC).

Nail changes in pemphigus: a systematic review and meta-analysis.

Pemphigus is a group of autoimmune mucocutaneous bullous disorders characterized by acantholysis resulting from autoantibodies targeting epithelial cell surface antigens. Studies reflect the presence of nail manifestations in some patients and suggest a potential correlation with clinical severity. This study examines the overall prevalence and characterizes the diverse manifestations of nail changes in pemphigus. We searched Cochrane, MEDLINE, EMBASE, and LILACS from 1990 to June 26, 2023 for studies reporting different nail changes in pemphigus patients. Data were collected and pooled to obtain proportions of the prevalence of nail changes in patients with pemphigus and subgroup analysis for pemphigus foliaceous and pemphigus vulgaris. The risk of bias was assessed with the Joanna Briggs Institute Checklist. Of 321 studies screened, 14 studies with 1,208 patients were included. Paronychia (n = 185) and Beau's lines (n = 104) were the most common nail changes identified. The pooled prevalence of nail disease in pemphigus patients was 0.389 (number of studies; [95% CI]: n = 9; [0.160-0.680], with high heterogeneity between studies (I2 = 95.0%, P < 0.001). Subgroup analysis revealed the highest prevalence in pemphigus foliaceous at 0.342 (n = 3; [0.109-0.688]) and pemphigus vulgaris at 0.396 (n = 5; [0.114-0.769]). Nail changes exhibited varied temporal relationships with disease onset and flares, preceding, concurrent, or following these events. Correlation with disease severity was noted, although discrepancies between studies were reported. Nail changes in pemphigus, particularly pemphigus vulgaris and pemphigus foliaceous, may be underrecognized. Observations regarding temporal associations and potential correlations with disease severity highlight the diagnostic and prognostic implications of nail changes in pemphigus. The limitations of this study include study heterogeneity and possible bias. Further research to establish the correlation of the presence and severity of nail changes on the overall disease course would be helpful.

Development and validation of the Dermatology Social Comparison (DSC) Scale.

BACKGROUND: Social comparison, the process of evaluating one's characteristics in relation to others, influences individuals' self-perception and behavior. However, instruments are scarce for assessing social comparison in the medical setting. OBJECTIVES: Our aim was to develop and validate a new scale for assessing social comparison. MATERIALS AND METHODS: Seven statements were developed, encompassing the perceived normality of having rashes, the tendency to compare their situation with others, and the emotional response when seeing someone better or worse off than themselves. The instrument was piloted in 15 patients for readability and face validity, then prospectively validated using modern psychometric methods in 1,053 adult patients with eczema or psoriasis from three tertiary dermatological centers in Singapore. RESULTS: Of 1,053 adult patients, 802 (76.2%) had eczema, and 251 (23.8%) had psoriasis. Exploratory factor analysis (using a 70% sample split) showed a single factor model comprising three questions (Eigenvalue: 1.4). Confirmatory factor analysis with the remaining 30% of the sample confirmed an excellent model fit. Cronbach's alpha was 0.7, and inter-item correlations ranged from 0.42 to 0.46. In the Rasch analysis, item fit statistics and item characteristic curves showed appropriate discrimination between response options, although reliability was suboptimal with a person separation reliability of 0.63. CONCLUSIONS: Comprising 3 questions, the newly derived social comparison scale showed acceptable psychometrics as a measure of social comparison for clinical and research purposes in dermatology. Its brief nature likely results from its brevity and applicability to conditions beyond eczema and psoriasis, which warrants further investigation.

The University of Texas Houston Stroke Registry (UTHSR): implementation of enhanced data quality assurance procedures improves data quality.

BACKGROUND: Limited information has been published regarding standard quality assurance (QA) procedures for stroke registries. We share our experience regarding the establishment of enhanced QA procedures for the University of Texas Houston Stroke Registry (UTHSR) and evaluate whether these QA procedures have improved data quality in UTHSR. METHODS: All 5093 patient records that were abstracted and entered in UTHSR, between January 1, 2008 and December 31, 2011, were considered in this study. We conducted reliability and validity studies. For reliability and validity of data captured by abstractors, a random subset of 30 records was used for re-abstraction of select key variables by two abstractors. These 30 records were re-abstracted by a team of experts that included a vascular neurologist clinician as the "gold standard". We assessed inter-rater reliability (IRR) between the two abstractors as well as validity of each abstractor with the "gold standard". Depending on the scale of variables, IRR was assessed with Kappa or intra-class correlations (ICC) using a 2-way, random effects ANOVA. For assessment of validity of data in UTHSR we re-abstracted another set of 85 patient records for which all discrepant entries were adjudicated by a vascular neurology fellow clinician and added to the set of our "gold standard". We assessed level of agreement between the registry data and the "gold standard" as well as sensitivity and specificity. We used logistic regression to compare error rates for different years to assess whether a significant improvement in data quality has been achieved during 2008-2011. RESULTS: The error rate dropped significantly, from 4.8% in 2008 to 2.2% in 2011 (P < 0.001). The two abstractors had an excellent IRR (Kappa or ICC ≥ 0.75) on almost all key variables checked. Agreement between data in UTHSR and the "gold standard" was excellent for almost all categorical and continuous variables. CONCLUSIONS: Establishment of a rigorous data quality assurance for our UTHSR has helped to improve the validity of data. We observed an excellent IRR between the two abstractors. We recommend training of chart abstractors and systematic assessment of IRR between abstractors and validity of the abstracted data in stroke registries.