Publications by authors named "Sarah D Cipriano"

Background: Significant gaps exist in the literature regarding the work-up and management of staphylococcal scalded skin syndrome (SSSS). These include (a) the utility of ancillary testing in improving diagnostic accuracy and informing patient care, and (b) appropriate management strategies for antibiotics, fluids, and skin care. Moreover, no systematic review to date has focused on non-neonatal pediatric SSSS to provide a comprehensive analysis of the literature.

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Background: This case report describes a 10-year-old male with a history of kidney transplantation who developed trichodysplasia spinulosa (TS), a rare skin disorder associated with immunosuppression.

Methods: The patient's condition, characterized by follicular papules and keratinous spines primarily on the face, was managed by a multidisciplinary team.

Results: Treatment involved reducing immunosuppression and administering topical cidofovir alongside oral leflunomide, leading to significant clinical improvement over several months.

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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.

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Article Synopsis
  • Ichthyoses are skin disorders marked by scaling and redness, leading to the creation of the Ichthyosis Scoring System (ISS) to quantify their severity effectively.
  • A study involving 65 participants found that ISS had excellent reliability for scoring skin conditions, both through live evaluation and photographic images.
  • The results indicate that ISS is a valid and reliable tool for assessing ichthyosis severity, making it suitable for clinical use and potentially a standard in the field.
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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.

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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).

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Article Synopsis
  • This review addresses updates related to common skin diseases in hospitalized children, focusing on their evaluation and management.
  • Notable conditions included are Staphylococcal scalded skin syndrome (SSSS), which is on the rise and mainly caused by MSSA, and toxic epidermal necrolysis (TEN), for which treatment options are still being debated.
  • The review also highlights multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19, emphasizing the importance of recognizing its dermatologic signs for timely diagnosis.
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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.

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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.

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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).

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Article Synopsis
  • - A case study details a 17-year-old female with linear porokeratosis, a skin condition characterized by abnormal keratinization and bone resorption, showing significant improvement with a treatment of 2% cholesterol/2% lovastatin ointment.
  • - Porokeratosis involves a skin layer (cornoid lamella) that includes incorrectly matured cells and can arise from mutations in the mevalonate pathway, suggesting a complex genetic basis for the disorder.
  • - This is one of the few cases documenting bone resorption related to linear porokeratosis, with only one previous case reported involving a distal digit.
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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.

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Article Synopsis
  • The COVID-19 pandemic prompted the formation of a Pediatric Dermatology COVID-19 Response Task Force to provide guidance on managing immunosuppressive therapies in children, due to a lack of evidence-based data about their safety during the pandemic.
  • A survey of 37 pediatric dermatology experts revealed that 97% altered their decisions about starting immunosuppressive medications because of the pandemic, with many pausing or reducing lab monitoring for these treatments.
  • Experts generally agreed on the importance of temporarily discontinuing medications for patients with COVID-19 exposure or positive tests, while also emphasizing that each case should be carefully considered in discussions with patients and their families.
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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 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.

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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.

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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.

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