Transition readiness in adolescents and young adults with chronic genetic skin conditions.

BACKGROUND/OBJECTIVES: Healthcare transition (HCT) refers to movement from pediatric to adult healthcare models. Lack of HCT preparation contributes to poor health outcomes. This study measures readiness to transition in individuals with genetic skin conditions. METHODS: Participants signed IRB-approved consents/assents. Participants ages 14-22 years with genetic skin disorders were surveyed with measures of QoL (Children's Dermatology Life Quality Index/CDLQI or DLQI) and HCT readiness using the Transition Readiness Assessment Questionnaire (TRAQ) and adapted non-validated measures of Skin Knowledge and Psychosocial Factors (5 = highest readiness). Mean TRAQ was compared with historical data on controls and other chronic conditions (t-tests) and correlated (Pearson) with Skin Knowledge and Psychosocial. Multivariable regression compared demographics and QoL with transition readiness. RESULTS: A total of 45 participants were enrolled (mean age 17.8 years, 67% female, 71% White; disorders of cornification [n = 31], ectodermal dysplasias [n = 7], epidermolysis bullosa [n = 4], tuberous sclerosis [n = 3]). Mean TRAQ (3.3 ± 0.9) was lower than controls (3.9; p < .001) and some chronic disorders (sickle cell [3.7; p < .05], type 1 diabetes [3.7; p < .01]), but higher than with spina bifida (2.8; p < .001) and congenital heart disease (2.9; p < .01). Mean Skin Knowledge was 4.2 ± 1.0, and mean Psychosocial was 3.4 ± 0.8. TRAQ correlated strongly with Skin Knowledge (r = .61; p < .05), but not Psychosocial (r = .12; p = .6). Ages 14-17 years versus 18-22 years and public versus private insurance predicted lower TRAQ scores. Poor DLQI predicted higher TRAQ and Skin Knowledge, but poor DLQI and CDLQI predicted lower Psychosocial. CONCLUSIONS: Adolescents and young adults with genetic skin disorders demonstrated low transition readiness, especially among younger-aged and lower socioeconomic groups. We recommend a HCT intervention to improve health outcomes.

Non-pulmonary Disseminated Tuberculosis Complicated by Constrictive Pericarditis and Cutaneous Gumma.

A 23-year-old previously healthy male presented to the hospital with symptoms of heart failure. He was diagnosed with pericarditis and found to have a reduced left ventricular ejection fraction of 25%. He was noted to have mediastinal lymphadenopathy. Pulmonary and abdominal sampling were non-diagnostic for infection, autoimmune disease, or malignancy. A QuantiFERON Gold returned positive. After a thorough travel history and detailed exam, the patient was diagnosed with disseminated tuberculosis after the discovery of a cutaneous gumma that was found to have acid-fast bacilli present on biopsy with Fite's stain. 18F-FDG PET CT and cardiac MRI were pursued given that pericardial and myocardial biopsy could not be safely performed due to the patient's hemodynamics. 18F-FDG PET CT and cardiac MRI did not demonstrate any myocardial pathology responsible for the left ventricular ejection fraction. This case highlights that pulmonary involvement is not necessary for disseminated TB, Fite's stain may be used to identify M. tuberculosis, and that cardiac MRI and 18F-FDG PET CT may be useful to delineate myocardial involvement in high-risk situations.

Myths and media in oral collagen supplementation for the skin, nails, and hair: A review.

BACKGROUND: As a key component of the hair, skin, and nails, there is strong consumer interest in the dermatologic efficacy of oral collagen supplementation. Oral supplementation with collagen peptides has increased in popularity in recent years. AIMS: There are relatively few studies investigating the dermatologic effects of ingested collagen peptides, many of which are limited by sample size and variability of results. The question remains whether there is sufficient evidence to support companies' promises and consumers' goals. METHODS: In this review, we investigate and compare the claims surrounding collagen supplementation on Instagram and YouTube, made by collagen companies, and established in the literature. RESULTS: Although some studies have demonstrated that collagen supplementation can enhance skin qualities such as elasticity and hydration, dermatologic claims in the media surpass any evidence currently supported by the literature. CONCLUSIONS: More research is needed to establish knowledge of the effects and physiologic mechanism of collagen supplementation. Dermatologists should be aware of the unsubstantiated proclamations of collagen made by companies and in social media, as well as what evidence is established thus far, to be equipped to discuss collagen supplementation with patients.

The Role of Diet Modification in Atopic Dermatitis: Navigating the Complexity.

Diet has long been understood to have an intricate association with atopic dermatitis, although much remains unelucidated. Skin barrier dysfunction with dysbiosis and consequent impairment of immune tolerance likely underly the pathogenesis of coincident atopic dermatitis and food allergy. There is a wide range of possible skin reactions to food, complicating the diagnosis and understanding of food allergies. Many patients, parents, and providers incorrectly suspect diet as causative of atopic dermatitis symptoms and many have tried elimination diets. This frequently leads to inaccurate labeling of food allergies, contributing to a dangerous spiral of inappropriate testing, referrals, and dietary changes, while neglecting established atopic dermatitis treatment essentials. Alternatively, certain dietary supplements or the introduction of certain foods may be beneficial for atopic dermatitis management or prevention. Greater consensus on the role of diet among providers of patients with atopic dermatitis is strongly encouraged to improve the management of atopic dermatitis.

Incorporating joint pain screening into the pediatric dermatologic examination.

BACKGROUND/OBJECTIVES: Joint manifestations are a feature of many pediatric skin disorders, among them psoriasis, autoimmune and autoinflammatory diseases, hypermobility disorders, and as an adverse effect of certain medications. Identifying joint disease early is important for intervention and prevention of chronic damage. However, pediatric musculoskeletal complaints are common and determining whether symptoms warrant a rheumatology referral for arthritis can be challenging. METHODS: Pediatric dermatologists were surveyed for their comfort in screening for joint disease. Through literature review and interviews with three pediatric rheumatologists, key joints involved in disorders with skin manifestations and arthritis were identified and the essential evaluations were determined. RESULTS: Of 100 surveyed practicing board-certified pediatric dermatologists, 79% did not feel confident in their ability to perform a joint-focused physical examination, a key step in screening for joint disease. A rapid joint examination technique (R-JET) was developed, along with an accompanying three-question survey and body diagram for patient self-report of symptoms. A video demonstration of the R-JET was created as a teaching tool. CONCLUSIONS: Teaching and incorporation of a rapid screening examination for arthritis by pediatric dermatologists has the potential to identify pediatric arthritis earlier, facilitate referral, and reduce the risk of progressive joint disease. These instruments can easily be incorporated into a pediatric dermatology office visit.

pH-Activated Single Molecule Conductance and Binding Mechanism of Imidazole on Gold.

We identify imidazole as a pH-activated linker for forming stable single molecule-gold junctions with several distinct configurations and reproducible electrical characteristics. Using a scanning tunneling microscope break junction (STMBJ) technique, we find multiple robust conductance signatures at integer multiples of 1.9 × 10-2G0 and 1.2 × 10-4G0 and determine that this molecule bridges the electrodes in its deprotonated form through the nitrogen atoms in basic conditions only, with several molecules able to bind in parallel and in series. The elongation these junctions can sustain is longer than the length of the molecule, suggesting that plastic deformation of gold electrodes occurs during stretching. Density functional theory calculations confirm that the imidazolate-linked junctions exhibit bond strengths of ∼2 eV, which can allow for plastic extraction of gold atoms. On the basis of these results, we hypothesize that lower conductance peaks correspond to chains of repeating molecule-gold units that we form and measure in situ.