In-person validation of the Ichthyosis Scoring System.

BACKGROUND: Ichthyoses are a heterogeneous group of skin disorders characterized by scaling and erythema. Recognizing the variability of scale and erythema by region and ichthyosis subtype, we developed the Ichthyosis Scoring System (ISS) to quantify severity. We previously found ISS to have high inter- and intrarater reliability in evaluating photographic images. To confirm ISS clinical utility, we examined its performance at the 2022 Foundation for Ichthyosis and Related Skin Types conference. METHODS: Sixty-five participants were evaluated by 3 of 9 medical professionals trained to score ichthyosis scale and erythema using ISS. Intrarater and interrater intraclass correlation coefficients (ICC) were analyzed using one-way and two-way random effects models, respectively. RESULTS: Intrarater reliability was excellent (ICC = 0.931, 95% CI, 0.921-0.940) for scale and good (ICC = 0.876, 95% CI, 0.853-0.899) for erythema scoring. Compared to photo validation with excellent intrarater reliability ratings for both scale (ICC = 0.956, 95% CI, 0.925-0.974) and erythema (ICC = 0.913, 95% CI, 0.855-0.949), ISS demonstrated equivalent reliability for live use. Overall interrater reliability for 10 body sites showed excellent (ICC >0.9) and good (ICC >0.75) agreement and consistency for both scale and erythema. Palms were an exception, demonstrating moderate (ICC >0.5) interrater agreement and consistency for erythema evaluation. CONCLUSIONS: ISS is a reliable measure of global and regional ichthyosis severity during in-person evaluations. Ease-of-use, accessibility, and content validity in both live and photographic evaluation endorse ISS as a standard for ichthyosis severity analysis.

Pediatric consultative dermatology: A survey of the Society for Pediatric Dermatology workforce reveals shortcomings in existing practice models of pediatric dermatology consult services in the United States.

The rate of pediatric hospitalization for cutaneous pathology has been increasing in recent years, often requiring the expertise of consulting pediatric dermatologists; however, the infrastructure of inpatient pediatric dermatology consultative services remains poorly characterized. We sought to assess the structure, consult volume, physician compensation, and utilization of teledermatology in pediatric dermatology inpatient services to better understand the current care model. Our survey of 118 pediatric dermatologists revealed that 89% of respondents see between 1 and 10 new consults per week, 39% perform all inpatient consults including evening and weekends without assistance from other providers, 71% do not have protected time during the week to provide inpatient consultations, and only 10% receive financial compensation via stipend. By highlighting both the high demand for pediatric consultative dermatology as well as the significant burden placed on these providers by existing practice models, we hope to encourage a reappraisal of the current infrastructure of pediatric inpatient dermatology to increase structural and financial support for this vital service.

Skin Color Representation in Teaching Photographs: One Institution's Approach to Addressing Visual Racism in Medical Education.

Problem: Visual racism refers to both the underrepresentation and inappropriate representation of darker skin types in medical education. By not teaching medical students and resident physicians to recognize common conditions in darker skin, it perpetuates biases that contribute to healthcare disparities for racial and ethnic minoritized groups. In this paper we describe our efforts to engage in institutional anti-racism work by addressing imbalances in representation of darker skin types in visual teaching images within our institution's curriculum. Intervention: We initially surveyed preclinical medical students regarding their perceptions of skin color representation in two courses. Researchers recorded the skin types of all teaching photographs in these courses in 2020. We then provided feedback and education to faculty, proposing that they increase brown and black skin color representation in educational content. During 2021, we reviewed the same courses and surveyed students again to ascertain the implementation and impact of our proposal. Context: We applied our intervention to two courses, Host & Defense (H&D) and Skin, Muscle, Bone, and Joint (SMBJ) since both courses utilize a large number of teaching images. Impact: From 2020 to 2021, both H&D and SMBJ significantly increased the proportion of visual teaching images that included darker skin types, with an increase from 28% to 42% in H&D and 20% to 30% in SMBJ. Significantly more students in the courses' 2021 iterations (73% in H&D, 93% in SMBJ) felt that lectures had appropriate representations of darker skin types when compared to students who took the course in 2020 (8% in H&D, 51% in SMBJ). Students in 2021 felt more confident in recognizing dermatological signs and symptoms in patients with darker skin than students in 2020. The majority of students in both 2020 and 2021 reported wanting to see a gradient of skin types for every dermatological condition discussed. Lessons learned: Our work suggests that addressing visual racism can be achieved partly by setting expectations for increased visual representation, collaborating across educational departments, and establishing clear metrics for assessing implementation. Future interventions will require a continual feedback loop of monitoring learning material, assessing faculty and student perception, refining resources, and recommending revisions to improve visual representation across the entire curriculum.

Structured Case-Based Ethics Discussion for Trainees and Faculty on Dermatopathology.

Introduction: Ethical and professional dilemmas are part of the day-to-day practice of medicine, including within dermatopathology (e.g., ethical implications of self-referring skin biopsies for pathology interpretation). There is a need for teaching aids that dermatology educators can easily access to help provide ethics education. Methods: We held an hour-long, faculty-facilitated, interactive, virtual discussion about ethical issues in dermatopathology. The session followed a structured, case-based format. We administered anonymous online feedback surveys after the session and used the Wilcoxon signed rank test to compare participants' before and after responses. Results: Seventy-two individuals from two academic institutions participated in the session. We collected 35 total responses (49%) from dermatology residents (n = 15), dermatology faculty (n = 14), medical students (n = 2), and other providers and learners (n = 4). Feedback was largely positive, with 21 attendees (60%) indicating they learned a few things and 11 (31%) indicating they learned a great deal. Additionally, 32 participants (91%) indicated they would recommend the session to a colleague. Our analysis showed that attendees had a greater self-perceived level of achievement for each of our three objectives after the session. Discussion: This dermatoethics session is structured so as to be easily shared, deployed, and built on by other institutions. We hope that other institutions will use our materials and results to improve upon the foundation presented here and that this framework will be used by other medical specialties seeking to foster ethics education in their training programs.

Evolving trends in inpatient paediatric dermatology.

PURPOSE OF REVIEW: This review provides updates in the evaluation and management of key dermatologic diseases encountered in the hospitalized child. RECENT FINDINGS: Our understanding of dermatologic disorders in children continues to evolve. Staphylococcal scalded skin syndrome (SSSS) is a potentially severe blistering disorder typically seen in children under the age of 4 with an incidence that is increasing in the United States. Recent research has highlighted that the majority of cases are due to methicillin-sensitive Staphylococcus aureus (MSSA) and most patients can be adequately managed with beta-lactams. Toxic epidermal necrolysis (TEN) is one of the most feared dermatologic disorders. Currently, there is a lack of consensus on the most efficacious first-line systemic therapy. Etanercept is increasingly being used based on studies showing a shorter time to re-epithelization and decreased mortality. Lastly, the COVID-19 pandemic introduced the novel inflammatory condition multisystem inflammatory syndrome in children (MIS-C) in which approximately three out of four children present with a mucocutaneous eruption. Early recognition of the dermatologic features of MIS-C is important in potentially establishing a diagnosis and differentiating it from the many other causes of childhood fever and rash. SUMMARY: There are no clear universal treatment guidelines for these rare diagnoses, and therefore, clinicians must remain informed of the latest findings in diagnosis and therapeutics.

Staphylococcal scalded skin syndrome: Clinical features, ancillary testing, and patient management.

BACKGROUND/OBJECTIVES: The utility of ancillary testing in improving diagnostic precision or improving patient outcomes in staphylococcal scalded skin syndrome (SSSS) is unclear. Similarly, an optimal antibiotic regimen has yet to be established. Our goal was to describe clinical characteristics and ancillary work-up of SSSS, report bacterial resistance patterns, and examine patient outcomes under varying therapeutic strategies with the aim of developing an evidence-based management algorithm. METHODS: We performed a retrospective review of pediatric patients diagnosed with SSSS at Intermountain Healthcare facilities between 2010 and 2021. A Kruskal-Wallis rank sum test was used to assess median length of stay between different antibiotic regimens. RESULTS: Eighty-five cases were identified. The most common ancillary tests obtained were a complete blood count (88%), followed by chemistry analysis (80%). Blood cultures were collected in more patients (79%) compared to aerobic cultures (60%). No blood culture was positive for Staphylococcus aureus. All S. aureus isolates were methicillin-sensitive. Of those found resistant to clindamycin (36%), all demonstrated macrolide-induced clindamycin resistance. None were constitutively resistant to clindamycin. There was no statistical difference between antibiotic regimen and length of stay (p = .691). Receiving opiate medications was the only risk factor associated with prolonged hospitalization (p = .001). CONCLUSIONS: Ancillary testing does not improve diagnostic precision and can be reduced. Clindamycin does not improve patient outcomes, suggesting beta-lactams should be considered first line. Susceptibility patterns in our cohort demonstrate inducible clindamycin resistance as opposed to constitutive.

Morbilliform Eruptions in the Hospitalized Child.

Morbilliform eruptions inspire a broad and varied differential spanning across inflammatory and infectious categories. The goal of this article is to help the clinician develop an approach toward the pediatric patient with a morbilliform eruption in the emergency room or hospital setting. The authors review several high-yield clinical scenarios with a focus on recently emerging and reemerging childhood diagnoses.

Neonatal pemphigoid gestationis: An atypical presentation of a rare disease.

Bullous pemphigoid (BP) is an autoimmune blistering disease characterized by urticarial plaques and/or vesicles and tense bullae. A unique presentation of BP can occur during pregnancy, the postpartum period after delivery, or with the initiation of contraception, in which case it is referred to as pemphigoid gestationis (PG). In rare instances, newborns born to mothers with PG may also present with blisters due to transplacental passage of maternal anti-bullous pemphigoid 180 (BP180) or 230 (BP230) immunoglobulin G (IgG). In this report, we present an unusual case of neonatal PG in an infant born to an asymptomatic mother without a previous diagnosis of PG.

Linear porokeratosis with bone abnormalities treated with compounded topical 2% cholesterol/2% lovastatin ointment.

We describe a case of linear porokeratosis with associated bone resorption in a 17-year-old female with marked improvement after 2% cholesterol/2% lovastatin ointment application. Porokeratosis is a heterogenous group of keratinization disorders characterized by a cornoid lamella, consisting of focal dyskeratotic cells in the granular layer and columns of parakeratosis. The pathogenesis of porokeratosis is not fully elucidated; however, germline mutations have recently been identified in the mevalonate pathway which can lead to a buildup of metabolites that could play a role in dysmaturation. There has only been one prior report of an affected distal digit with underlying bone resorption in association with linear porokeratosis.

Chondrodysplasia punctata and neonatal lupus in an infant with positive anti-RNP and negative anti-Ro/SSA and -La/SSB antibodies, a case report.

Rhizomelic chondrodysplasia punctata is a rare, often fatal disease that shares many clinical dysmorphologic features with the rare often non-lethal chondrodysplasia punctata due to maternal autoimmune disease. Characteristic findings of both conditions include mid-face hypoplasia, stippled epiphyses of the vertebrae and long bones, and growth failure. A growing association with anti-ribonucleoprotein antibodies is emerging amongst patients with chondrodysplasia punctata due to maternal autoimmune disease and also neonatal lupus that have potential important screening implications. We present a unique case of chondrodysplasia punctata with neonatal lupus in the setting of positive anti-RNP antibodies and negative anti-Ro/SSA and -La/SSB antibodies born to a mother with mixed connective tissue disease and Raynaud's syndrome.

Systemic immunosuppressive therapy for inflammatory skin diseases in children: Expert consensus-based guidance for clinical decision-making during the COVID-19 pandemic.

BACKGROUND/OBJECTIVES: The COVID-19 pandemic has raised questions about the approach to management of systemic immunosuppressive therapies for dermatologic indications in children. Change to: Given the absence of data to address concerns related to SARS-CoV-2 infection and systemic immunosuppressive therapies in an evidence-based manner, a Pediatric Dermatology COVID-19 Response Task Force (PDCRTF) was assembled to offer time-sensitive guidance for clinicians. METHODS: A survey was distributed to an expert panel of 37 pediatric dermatologists on the PDCRTF to assess expert opinion and current practice related to three primary domains of systemic therapy: initiation, continuation, and laboratory monitoring. RESULTS: Nearly all respondents (97%) reported that the COVID-19 pandemic had impacted their decision to initiate immunosuppressive medications. The majority of pediatric dermatologists (87%) reported that they were pausing or reducing the frequency of laboratory monitoring for certain immunosuppressive medications. In asymptomatic patients, continuing therapy was the most popular choice across all medications queried. The majority agreed that patients on immunosuppressive medications who have a household exposure to COVID-19 or test positive for new infection should temporarily discontinue systemic and biologic medications, with the exception of systemic steroids, which may require tapering. CONCLUSIONS: The ultimate decision regarding initiation, continuation, and laboratory monitoring of immunosuppressive therapy during the pandemic requires careful deliberation, consideration of the little evidence available, and discussion with families. Consideration of an individual's adherence to COVID-19 preventive measures, risk of exposure, and the potential severity if infected must be weighed against the dermatological disease, medication, and risks to the patient of tapering or discontinuing therapies.

Severe Facial Herpes Vegetans and Viremia in NFKB2-Deficient Common Variable Immunodeficiency.

With the accessibility of next-generation sequencing modalities, an increasing number of primary immunodeficiency disorders (PIDDs) such as common variable immunodeficiency (CVID) have gained improved understanding of molecular pathogenesis and disease phenotype with the identification of a genetic etiology. We report a patient with early-onset CVID due to an autosomal dominant loss-of-function mutation in NFKB2 who developed a severe herpes vegetans cutaneous infection as well as concurrent herpes simplex virus viremia. The case highlights features of CVID, unique aspects of NF-κB2 deficiency including susceptibility to herpesvirus infections, the detection of neutralizing anticytokine antibodies, and the complexity of medical management of patients with a PIDD that can be aided by a known genetic diagnosis.

Localization and age distribution of telangiectases in children and adolescents with hereditary hemorrhagic telangiectasia: A retrospective cohort study.

BACKGROUND: The location of telangiectases in hereditary hemorrhagic telangiectasia (HHT), as set forth in the consensus diagnostic (Curaçao) criteria, is based primarily on adults. OBJECTIVE: Document the locations and numbers of telangiectases in a cohort of pediatric patients with HHT. METHODS: A retrospective chart review using a standardized data collection form for site and number of telangiectases was performed for pediatric patients with HHT (age, 0-18 years) from 2005 to 2016. RESULTS: Of 90 pediatric patients with HHT, 71% had one or more telangiectases. Of all the telangiectases counted (N = 319), cutaneous telangiectases were more common (73%) than oral telangiectases (27%). The hands were the most frequent site, accounting for 33% of all telangiectases. Adolescents were more likely than children to have cutaneous telangiectases (85% vs 50% [Q = 0.005]). The most frequent sites in children younger than 10 years were the hands excluding the fingers (27%), fingers (25%), and face (23%). Only 23% of subjects (21 of 90) presented with multiple (≥3) telangiectases at locations considered characteristic for the current consensus diagnosis guidelines (lips, oral cavity, and fingers). LIMITATIONS: Ascertainment bias based on recruitment. CONCLUSIONS: In this pediatric population, telangiectases at sites not included as "characteristic" by the Curaçao diagnostic criteria were common. The Curaçao criteria in regard to both number and location of telangiectases may be inadequate in the pediatric HHT population.

Online learning in a dermatology clerkship: piloting the new American Academy of Dermatology Medical Student Core Curriculum.

BACKGROUND: Multiple studies have shown that both current and future primary care providers have insufficient education and training in dermatology. To address the limitations and wide variability in medical student dermatology instruction, the American Academy of Dermatology (AAD) created a standardized, online curriculum for both dermatology learners and educators. OBJECTIVE: We sought to determine the impact of the integration of the AAD online curriculum into a 2-week introductory dermatology clerkship for fourth-year medical students. METHODS: In addition to their clinical duties, we assigned 18 online modules at a rate of 1 to 3 per day. We evaluated knowledge acquisition using a 50-item, multiple-choice pretest and posttest. Postmodule and end-of-course questionnaires contained both closed and open-ended items soliciting students' perceptions about usability and satisfaction. RESULTS: All 51 participants significantly improved in their dermatology knowledge (P < .001). The majority of students found the modules easy to navigate (95%) and worth their time (93%). All respondents supported the continuation of the modules as part of the dermatology clerkship. LIMITATIONS: Without a control group who did not experience the online curriculum, we are unable to isolate the specific impact of the online modules on students' learning. CONCLUSION: This study demonstrates the successful integration of this educational resource into a 2-week, university-based dermatology clerkship. Students' perceptions regarding usability and satisfaction were overwhelmingly positive, suggesting that the online curriculum is highly acceptable to learners. Widespread use of this curriculum may be a significant advancement in standardized dermatology learning for medical students.