Severe congenital ichthyosiform dermatosis in CHIME syndrome successfully treated with ixekizumab.

Coloboma, congenital heart disease, ichthyosiform dermatosis, intellectual disability, conductive hearing loss, and epilepsy (CHIME) syndrome is a rare autosomal recessive neuroectodermal disorder caused by PIGL gene mutations. There is emerging literature to support the use of interleukin-17 (IL-17) antagonists in the treatment of certain ichthyosiform dermatoses. Here, we report a case of severe ichthyosiform dermatosis in a child with CHIME syndrome who was recalcitrant to multiple topical medications and dupilumab.

Antiviral drugs prolong survival in murine recessive dystrophic epidermolysis bullosa.

Recessive dystrophic epidermolysis bullosa (RDEB) is a rare inherited skin disease characterized by defects in type VII collagen leading to a range of fibrotic pathologies resulting from skin fragility, aberrant wound healing, and altered dermal fibroblast physiology. Using a novel in vitro model of fibrosis based on endogenously produced extracellular matrix, we screened an FDA-approved compound library and identified antivirals as a class of drug not previously associated with anti-fibrotic action. Preclinical validation of our lead hit, daclatasvir, in a mouse model of RDEB demonstrated significant improvement in fibrosis as well as overall quality of life with increased survival, weight gain and activity, and a decrease in pruritus-induced hair loss.

Closing the gap: Enhancing quality pediatric dermatologic care through project Extension for Community Healthcare Outcomes (ECHO).

This study focused on evaluating Extension for Community Healthcare Outcomes (ECHO) participating primary care clinician's (PCC's) diagnostic and treatment accuracy of pediatric dermatologic conditions. To evaluate this, pediatric cases presented to Dermatology ECHO by PCCs with questions regarding diagnosis, treatment regimen, or both were analyzed. After PCC case presentation, the hub team of dermatologists facilitated case-based discussion and provided the presenter with mentorship and guidance regarding diagnosis and treatment of their patient.

JAK Inhibitors: A New Weapon in the Skin Care Providers' Arsenal.

A number of chronic skin diseases, such as vitiligo and alopecia areata, are historically resistant to or respond poorly to treatment. Additionally, disorders such as atopic dermatitis and psoriasis have subtypes that are inadequately treated by current medications. Lastly, in the field of dermatology there are a number of conditions, some genetic (such as Darier's disease and Hailey-Hailey disease) and others caused by aberrant inflammatory responses (macrophage-driven conditions such as sarcoidosis and autoimmune conditions such as localized scleroderma) where effective treatments have been limited to date.

Leprosy (Hansen's disease): An Update and Review.

Leprosy (Hansen's disease) is caused by infection with bacilli of the complex. It is considered an exotic and rare diagnosis in Missouri. Past leprosy patients diagnosed locally have typically acquired it in areas of the world where leprosy is endemic.

Assessing and optimizing readability of dermatology patient education materials (PEMs).

Patient education materials (PEMs) are a powerful tool to improve patient understanding; however, inadequate health literacy is a well-established barrier for PEMs to serve their purpose. The average American reads at an 8th grade level and the National Institute of Health (NIH) recommendation for PEMs is at the 6th grade level. The purpose of this study was to assess and optimize PEMs to identify changes that are most effective at lowering the reading level without diluting its educational content.

Climate change and pediatric skin health.

Children are known to disproportionately bear the health impacts of climate change, particularly children living in impoverished areas. Owing to their developing physiology and immature metabolism, distinct exposure behaviors, and reliance on adults for care and protection, children are uniquely susceptible to the adverse effects of our warming planet. Herein, we summarize the known impacts of climate change on pediatric skin health, including its effects on atopic dermatitis, vector-borne and other infectious diseases, nutritional deficiencies, and psychodermatoses.

Genodermatoses with malignant potential.

The hereditary nature of some forms of cancer was recognized long ago. Over time, recognition of associated findings led to the delineation of numerous hereditary cancer syndromes. Many of these syndromes also have cutaneous manifestations, the recognition of which can lead to their early identification.

Accelerating Care Through ECHO: Case Examples from the Field.

In this article, we describe three life-changing patient cases demonstrating high-quality and timely care they received in their communities, thanks to the Show-Me ECHO project. Early autism diagnosis, a potentially deadly tumor manifesting as a benign-looking rash, a recalcitrant case of hepatitis C: rural and underserved Missourians now have access to state-of-the-art care through their local providers receiving interdisciplinary telementoring on evidence based practices.

Cutaneous Drug Eruptions in Pediatrics-A Primer.

Cutaneous adverse drug reactions (ADRs) are commonly seen in the pediatric population in both inpatient and outpatient settings and are important to identify, evaluate, and appropriately manage. Early recognition and proper classification of a cutaneous drug reaction allows the clinician the ability to narrow in on a culprit drug and determine whether the medication is safe to continue. This review discusses the clinical presentation, categorization, and management of cutaneous ADRs in the pediatric population.

Utility of Dermatology Extension for Community Healthcare Outcomes (ECHO) sessions in the adult and paediatric population.

Introduction: Primary care provider (PCP) competency in dermatology is inadequate despite the high volume of patients with skin conditions. Better education and access to dermatology expertise is vital to improve patient care. We present a comprehensive case-based evaluation of Dermatology Extension for Community Healthcare Outcomes ECHO) sessions, an innovative videoconferencing educational model, by determining the diagnostic and treatment accuracy of dermatological conditions by PCPs over a 2-year period.

Technical and Imaging Outcomes from the UK Registry of Prostate Artery Embolization (UK-ROPE) Study: Focusing on Predictors of Clinical Success.

Introduction: The UK Registry of Prostate Artery Embolization (UK-ROPE) was a prospective, multicentre study comparing PAE against surgical therapies for symptomatic benign prostatic hyperplasia (BPH). A wealth of data was collected supplementary to the main study outcomes which provide a snapshot of UK PAE practice. We aimed to interpret these data in the hope of providing insight into factors which affect clinical outcome and radiation dose.

Experimental BPA Exposure and Glucose-Stimulated Insulin Response in Adult Men and Women.

Context: Human cross-sectional and animal studies have shown an association of the chemical bisphenol A (BPA) with insulin resistance, type 2 diabetes, and other metabolic diseases, but no human experimental study has investigated whether BPA alters insulin/C-peptide secretion.

Design: Men and postmenopausal women (without diabetes) were orally administered either the vehicle or a BPA dose of 50 µg/kg body weight, which has been predicted by US regulators (Food and Drug Administration, Environmental Protection Agency) to be the maximum, safe daily oral BPA dose over the lifetime. Insulin response was assessed in two cross-over experiments using an oral glucose tolerance test (OGTT; experiment 1) and a hyperglycemic (HG) clamp (experiment 2).

Uveitis Following Treatment of Verruca Vulgaris with Intralesional Antigen.

This is a first literature report of a case of uveitis along with severe systemic symptoms following verruca vulgaris treatment with intralesional antigen. We believe the injection was causative.

Prostate artery embolization: a new, minimally invasive treatment for lower urinary tract symptoms secondary to prostate enlargement.

Prostate artery embolization (PAE) is emerging as a safe and efficacious treatment which approaches benign prostatic obstruction (BPO) from a unique perspective. This brings with it distinct advantages and solutions, which we discuss along with cost, evidence, complications and disadvantages.

Efficacy and safety of prostate artery embolization for benign prostatic hyperplasia: an observational study and propensity-matched comparison with transurethral resection of the prostate (the UK-ROPE study).

Objectives: To assess the efficacy and safety of prostate artery embolization (PAE) for lower urinary tract symptoms (LUTS) secondary to benign prostatic hyperplasia (BPH) and to conduct an indirect comparison of PAE with transurethral resection of the prostate (TURP).

Patients And Methods: As a joint initiative between the British Society of Interventional Radiologists, the British Association of Urological Surgeons and the National Institute for Health and Care Excellence, we conducted the UK Register of Prostate Embolization (UK-ROPE) study, which recruited 305 patients across 17 UK urological/interventional radiology centres, 216 of whom underwent PAE and 89 of whom underwent TURP. The primary outcomes were International Prostate Symptom Score (IPSS) improvement in the PAE group at 12 months post-procedure, and complication data post-PAE.

Computationally constructing a repository of compound remote associates test items in American English with comRAT-G.

The Remote Associates Test (RAT) has been used to measure creativity, however few repositories or standardizations of test items exist, like the normative data on 144 items provided by Bowden and Jung-Beeman. comRAT is a computational solver which has been used to solve the compound RAT in linguistic and visual forms, showing correlation to human performance over the normative data provided by Bowden and Jung-Beeman. This paper describes using a variant of comRAT, comRAT-G, to generate and construct a repository of compound RAT items for use in the cognitive psychology and cognitive modeling community.

Planning Prostate Artery Embolisation: Is it Essential to Perform a Pre-procedural CTA?

Purpose: A highly variable prostatic artery origin coupled with frequent anastomoses to adjacent organs makes prostate artery embolisation a challenging procedure. Despite CT angiography facilitating procedural planning, it is not performed in all centres. Therefore, we explored the utility of prostatic CT angiography by assessing its capacity to identify the prostatic arteries and highlight troublesome anastomoses.

Cornoid Lamella-Like Structures in HIV-Associated Epidermodysplasia Verruciformis: A Unique Histopathologic Finding.

Epidermodysplasia verruciformis (EV) is an uncommon inherited skin condition with increased vulnerability to widespread infection by certain human papillomavirus types, resulting in extensive verruca plana-like papules coalescing to large confluent plaques. Since the AIDS epidemic starting in the 1980s, an acquired type of EV has been described in patients infected with human immunodeficiency virus. The histopathologic features of EV consist of papillated epidermal hyperplasia with hypergranulosis and a distinct bluish-gray color in the large human papillomavirus-infected keratinocytes in the stratum granulosum.

Extreme pain from brown recluse spider bites: model for cytokine-driven pain.

Importance: Bites from the brown recluse spider (BRS) can cause extreme pain. We propose cytokine release as a cause of the discomfort and a central mechanism through glial cell upregulation to explain measured pain levels and time course.

Observations: Twenty-three BRS bites were scored at a probable or documented level clinically, and an enzyme-linked immunosorbent assay was used to confirm the presence of BRS venom.