Rapid clearance of extensive juvenile pityriasis rubra pilaris with ixekizumab.

Juvenile pityriasis rubra pilaris is a rare inflammatory skin disorder currently without any FDA-approved treatments, and lesions can be refractory to conventional treatment with topical corticosteroids, methotrexate, and oral retinoids. We herein present a case of a 6-year-old boy who attained clearance of extensive juvenile pityriasis rubra pilaris within 2 weeks of starting ixekizumab therapy. Therapeutic effect has been durable at 6 months, and patient continues on therapy without adverse effects. Our case highlights a new, rapidly effective treatment option for pediatric patients with this rare condition.

Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder in children: A case report and review of the literature.

Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (PCSM-TCLPD) is a rare benign lymphoproliferative disorder, recently redefined by the 2016 World Health Organization classification of lymphoid neoplasms. In adults, PCSM-TCLPD responds well to monotherapy with surgical excision or local radiation, with or without topical/injected corticosteroids; in contrast, PCSM-TCLPD has only rarely been reported in children, in whom treatments favored in adults may be non-optimal. We present a 14-year-old male with PCSM-TCLPD on the forehead, who achieved complete remission following biopsy, topical corticosteroids, and surgical excision. We also review all literature-reported cases of pediatric PCSM-TCLPD, emphasizing the disorder's benign nature and treatment responsiveness in children.

Classification of lower extremity venous malformations and risk of knee involvement: A retrospective cohort study.

BACKGROUND: Venous malformation (VM) is the most common vascular anomaly in the lower extremity. VMs can be classified as focal, multifocal, or diffuse types. Intraarticular VM (IA-VM) of the knee portends morbidity. Association of the lower extremity VM type with IA-VM is not well defined. OBJECTIVE: To classify a large cohort of lower extremity, nonsyndromic VMs by type and determine associations with IA-VM. METHODS: Retrospective cohort study. RESULTS: We assessed 156 patients with nonsyndromic, lower extremity VM; 71 (46%) were focal and 85 (54%) were diffuse type VM, and 97 (62%) were IA-VM. Of diffuse VMs, 26 (31%) were Bockenheimer and 59 (69%) were localized subtypes. Pure VM had a significantly elevated risk of IA-VM (relative risk [RR], 2.34; 95% confidence interval [CI], 1.42-3.89). IA-VM was more common in diffuse (73%) versus focal (49%) types. Risk of IA-VM in diffuse type VM was significantly elevated (RR, 1.48; 95% CI, 1.13-1.94). One hundred percent of diffuse Bockenheimer type VM had IA-VM, and this subtype had the highest risk (RR, 1.83; 95% CI, 1.56-2.14) of IA-VM. LIMITATIONS: Retrospective, single-institution study. CONCLUSIONS: Intraarticular involvement of the knee should be considered in all lower extremity VMs. Pure VM and the Bockenheimer diffuse VM subtype had the highest risk of IA-VM.

Limited utility of repeated vital sign monitoring during initiation of oral propranolol for complicated infantile hemangioma.

BACKGROUND: Initial propranolol recommendations for infantile hemangioma published in 2013 were intended as provisional best practices to be updated as evidence-based data emerged. METHODS: A retrospective multicenter study was performed to evaluate utility of prolonged monitoring after first propranolol dose and escalation(s). Inclusion criteria included diagnosis of hemangioma requiring propranolol of greater than or equal to 0.3 mg/kg per dose, younger than 2 years, and heart rate monitoring for greater than or equal to 1 hour. Data collected included demographics, dose, vital signs, and adverse events. RESULTS: A total of 783 subjects met inclusion criteria; median age at initiation was 112 days. None of the 1148 episodes of prolonged monitoring warranted immediate intervention or drug discontinuation. No symptomatic bradycardia or hypotension occurred during monitoring. Mean heart rate change from baseline to 1 hour was -8.19/min (±15.54/min) and baseline to 2 hours was -9.24/min (±15.84/min). Three preterm subjects had dose adjustments because of prescriber concerns about asymptomatic vital sign changes. No significant difference existed in pretreatment heart rate or in heart rate change between individuals with later adverse events during treatment and those without. CONCLUSION: Prolonged monitoring for initiation and escalation of oral propranolol rarely changed management and did not predict future adverse events. Few serious adverse events occurred during therapy; none were cardiovascular.

Pediatric Pseudo-pseudoxanthoma Elasticum Resulting From D-Penicillamine Treatment for Wilson Disease.

We describe a 14-year-old boy with Wilson disease (WD) who first developed pseudo-pseudoxanthoma elasticum (PPXE) after 4.5 years of treatment with D-penicillamine. Although previously reported cases have occurred in adults following at least a decade of high-dose D-penicillamine use, this case demonstrates that D-penicillamine-induced PPXE can present in children with shorter treatment courses. Upon this diagnosis, the patient was switched from D-penicillamine to trientine, with adequate cupriuresis and stabilization of the skin lesion. Prompt diagnosis and management of PPXE in children can limit systemic progression and prevent long-term complications.

Pediatric facial pyoderma gangrenosum preceding the diagnosis of inflammatory bowel disease.

We describe two adolescent patients with pyoderma gangrenosum (PG) involving the face. Subsequent gastrointestinal evaluation revealed microscopic bowel inflammation suggestive of inflammatory bowel disease. While PG is rarely localized to the face, this brief report reveals two cases of pediatric facial PG and suggests a correlation between facial PG and microscopic colitis.

Soothing the heart with music: A feasibility study of a bedside music therapy intervention for critically ill patients in an urban hospital setting.

OBJECTIVES: Music therapy has been shown to be effective for reducing anxiety and pain in people with a serious illness. Few studies have investigated the feasibility of integrating music therapy into general inpatient care of the seriously ill, including the care of diverse, multiethnic patients. This leaves a deficit in knowledge for intervention planning. This study investigated the feasibility and effectiveness of introducing music therapy for patients on 4 inpatient units in a large urban medical center. Capacitated and incapacitated patients on palliative care, transplantation, medical intensive care, and general medicine units received a single bedside session led by a music therapist. METHODS: A mixed-methods, pre-post design was used to assess clinical indicators and the acceptability and feasibility of the intervention. Multiple regression modeling was used to evaluate the effect of music therapy on anxiety, pain, pulse, and respiratory rate. Process evaluation data and qualitative analysis of observational data recorded by the music therapists were used to assess the feasibility of providing music therapy on the units and patients' interest, receptivity, and satisfaction. RESULTS: Music therapy was delivered to 150 patients over a 6-month period. Controlling for gender, age, and session length, regression modeling showed that patients reported reduced anxiety post-session. Music therapy was found to be an accessible and adaptable intervention, with patients expressing high interest, receptivity, and satisfaction. SIGNIFICANCE OF RESULTS: This study found it feasible and effective to introduce bedside music therapy for seriously ill patients in a large urban medical center. Lessons learned and recommendations for future investigation are discussed.

Self-reported help-seeking behaviors and treatment choices of adolescents regarding acne.

Acne vulgaris is a common condition affecting adolescents that they often choose to treat on their own rather than seek out and follow medical advice. Using data from an anonymous survey administered to 1,214 students in public middle and high schools in New Jersey, we compared the self-reported acne frequency, severity, and beliefs of students based on their help-seeking behaviors, treatment choices, and treatment adherence. Chi-square analyses were performed for data comparison. A large proportion of students in this sample (57%) treated their own acne, and a much smaller proportion (17%) have sought medical care. Students who saw a health professional reported acne of higher frequency and severity than those who did not (p = 0.01). Severity also appeared to affect treatment adherence, with students who adhered to recommended treatments reporting more frequent (p < 0.001) and more severe (p = 0.02) acne than those who chose to self-treat. Beliefs and knowledge varied most significantly according to treatment adherence. In conclusion, most adolescent students treat their own acne. Self-assessment of acne severity plays a significant role in the tendency to seek out and adhere to medical treatment. Beliefs and knowledge may also affect adherence, suggesting a role for physicians to influence adherence rates through patient education. Because the majority of students are getting information from nonphysician sources, there may be a need to evaluate the resources they are using to make sure they are receiving appropriate, helpful information.

Hospital-Based Interventions to Address Provider Grief: A Narrative Review.

CONTEXT: Provider grief, i.e., grief related to the death of patients, often forms an ongoing and profound stressor impacting healthcare providers' ability to maintain their sense of well-being, avoid feeling overwhelmed, and sustain quality and compassionate patient care over time. OBJECTIVES: This narrative review presents findings on the types of interventions hospitals have offered to physicians and nurses to address provider grief. METHODS: Searches of PubMed and PsycINFO were conducted for articles (e.g., research studies, program descriptions and evaluations) focused on hospital-based interventions to help physicians and nurses cope with their own grief. RESULTS: Twenty-nine articles met inclusion criteria. The most common adult clinical areas were oncology (n = 6), intensive care (n = 6), and internal medicine (n = 3), while eight articles focused on pediatric settings. Nine articles featured education interventions, including instructional education programs and critical incident debriefing sessions. Twenty articles discussed psychosocial support interventions, including emotional processing debriefing sessions, creative arts interventions, support groups, and retreats. A majority of participants reported that interventions were helpful in facilitating reflection, grieving, closure, stress relief, team cohesion, and improved end-of-life care, yet mixed results were found related to interventions' effects on reducing provider grief to a statistically significant degree. CONCLUSION: Providers largely reported benefits from grief-focused interventions, yet research was sparse and evaluation methodologies were heterogenous, making it difficult to generalize findings. Given the known impact provider grief can have on the individual and organizational levels, it is important to expand providers' access to grief-focused services and to increase evidence-based research in this field.

Eruption of bullae within psoriatic plaques: a rare adverse effect of narrow-band ultraviolet B (NB-UVB) phototherapy.

The sudden eruption of bullae within psoriatic plaques is an uncommon adverse effect of narrow-band UVB phototherapy (TL-01 radiation). We report the case of a 49-year-old man who developed a bullous eruption after several NB-UVB treatments and will review important aspects of this unusual phototherapy complication.

Adverse Events and Safety of SARS-CoV-2 Vaccines: What's New and What's Next.

Just over 1 year following rollout of the first vaccines for coronavirus disease 2019, 572 million doses have been administered in the United States. Compared with the number of vaccines administered, adverse effects such as anaphylaxis have been rare, and seemingly, the more serious the effect, the rarer the occurrence. Despite these adverse effects, there are few, if any, true contraindications to coronavirus disease 2019 vaccination and most individuals recover without further sequelae. This review provides guidance for the allergist/immunologist regarding appropriate next steps based on patient's known allergy history or adverse reaction after receipt of coronavirus disease 2019 vaccine to assist in safe global immunization.

Aspirin-Exacerbated Respiratory Disease: A Unique Case of Drug Hypersensitivity.

This review of aspirin-exacerbated respiratory disease (AERD) describes the clinical characteristics and pathophysiology of disease, highlighting its similarities and unique differences in comparison to classic IgE mediated hypersensitivity as well as AERD as a chronic disease. There is a specific focus on the comparison of mediator production over time and the use of desensitization in each diagnosis that serves to aid the clinician in differentiating aspirin reactions in AERD from those related to true immediate hypersensitivity.

Using a qualitative approach to develop an evaluation data set for community-based health promotion programs addressing racial and ethnic health disparities.

Racial and ethnic disparities in health have increasingly become a central focus of health promotion efforts. At the community level, however, collecting data and evaluating these programs has been a challenge because of the diversity of populations, community contexts, and health issues as well as a range of capacities for conducting evaluation. This article outlines a qualitative research process used to develop a Web-based standard program performance data reporting system for programs funded by the U.S. Office of Minority Health (OMH), but generally applicable to community-based health promotion programs addressing health disparities. The "core-and-module" data set, known as the Uniform Data Set (UDS), is a Web-based system and is used as the programwide reporting system for OMH. The process for developing the UDS can be used by any agency, locality, or organization to develop a tailored data collection system allowing comparison across projects via an activity-based typology around which data reporting is structured. The UDS model enables the collection of grounded data reflecting community-level steps necessary to address disparities as well as a reporting structure that can guide data collection based on broader frameworks now emerging that specify criteria for measuring progress toward the elimination of health disparities.

COVID-19: Thrombosis, thromboinflammation, and anticoagulation considerations.

Vascular endothelial injury is a hallmark of acute infection at both the microvascular and macrovascular levels. The hallmark of SARS-CoV-2 infection is the current COVID-19 clinical sequelae of the pathophysiologic responses of hypercoagulability and thromboinflammation associated with acute infection. The acute lung injury that initially occurs in COVID-19 results from vascular and endothelial damage from viral injury and pathophysiologic responses that produce the COVID-19-associated coagulopathy. Clinicians should continue to focus on the vascular endothelial injury that occurs and evaluate potential therapeutic interventions that may benefit those with new infections during the current pandemic as they may also be of benefit for future pathogens that generate similar thromboinflammatory responses. The current Accelerating COVID-19 Therapeutic Interventions and Vaccines (ACTIV) studies are important projects that will further define our management strategies. At the time of writing this report, two mRNA vaccines are now being distributed and will hopefully have a major impact on slowing the global spread and subsequent thromboinflammatory injury we see clinically in critically ill patients.

Bedside Music Therapy for Women during Antepartum and Postpartum Hospitalization.

PURPOSE: Stress and anxiety are prevalent during pregnancy and postpartum with adverse effects on mothers and newborns, yet women's psychological and emotional needs are often given a lower priority than their physical wellbeing. The purpose of this study was to assess feasibility of implementing a bedside music therapy intervention to alleviate stress and anxiety, provide emotional support, and facilitate mother-baby bonding for women during antepartum and postpartum hospitalization at a large urban medical center. STUDY DESIGN AND METHODS: Over 15 months, women on three units who were hospitalized during antepartum or postpartum were referred for music therapy and received a single bedside session from a credentialed music therapist (MT-BC), including tailored interventions and education in relaxation techniques. A retrospective analysis of postintervention feedback questionnaires and process notes was conducted to assess participant receptivity and satisfaction, and the feasibility of implementing the program on the units. RESULTS: Music therapy was provided to 223 postpartum and 97 antepartum patients. The program was found to be feasible and well received, including high satisfaction, positive effects on participants' relaxation and sense of connection with their baby, and enthusiastic reception from providers and staff. Qualitative feedback revealed salient themes including the effect of the intervention on mothers' mental, emotional and physical states, and the soothing effect of music on their newborns. CLINICAL IMPLICATIONS: Hospitals are in a unique position to provide support services and self-care education for women during their antepartum and postpartum hospitalization. Music therapy can be integrated successfully into inpatient care as a nurturing and patient-centered form of psychosocial support.