Optimizing pediatric periprocedural pain management part I-Evolving ethics and topical anesthetics.

Pediatric procedure-related pain management is often incompletely understood, inadequately addressed, and critical in influencing a child's lifelong relationship with the larger health care community. We highlight the evolution of ethics and expectations around optimizing periprocedural pain management as a fundamental human right. We investigate the state-of-the-art of topical anesthetics, reviewing their mechanisms of action and providing comparisons of their relative safety and efficacy data to help guide clinical selection. In total, this two-part review offers a combination of conventional approaches and innovative techniques that should be used multimodally-in series and in parallel-to help optimize pain management and provide alternatives to sedation medication and general anesthesia.

Lived Experiences and Fear of Cancer Recurrence Among Survivors of Localized Cutaneous Melanoma.

Importance: Most of the rapid increase in cutaneous melanoma incidence in the US has been localized disease that is treated surgically and is associated with high survival rates. However, little is known about the psychological well-being of survivors in the US. Objective: To explore the lived experiences and fear of cancer recurrence among survivors of localized cutaneous melanoma. Design, Setting, and Participants: This was a qualitative and survey-based study that used semistructured interviews and the Fear of Cancer Recurrence Inventory short form (FCRI-SF) survey tool with participants recruited from an academic dermatology practice affiliated with the University of Texas, Austin. Interviews were completed via telephone or in person from August 2021 to September 2022. Each of the 9 items in the FCRI-SF was rated on a 5-point Likert scale, scored from 0 to 4, with a maximum possible score of 36 points. Data analyses were performed from February 2022 to June 2023. Main Outcomes and Measures: Semistructured interviews were analyzed for themes and subthemes associated with the lived experiences of survivors of cutaneous melanoma. The FCRI-SF scores were tabulated, with scores of 13 or greater identifying potential cases of clinically significant fear of cancer recurrence. Results: In all, 51 participants (mean [SD] age, 49.5 [11.7] years; 34 [67%] female and 17 [33%] male) with a history of localized melanoma (stage 0-IIA) completed the interview and survey. Among them, 17 (33%) had survived a diagnosis of stage 0 melanoma, and the remainder, at least 1 invasive melanoma diagnosis (stage I-IIA). Semistructured interviews revealed several themes: (1) emotions surrounding follow-up appointments, (2) intensity of melanoma surveillance, (3) lifestyle changes regarding sun exposure, and (4) thoughts about life and death. Thirty-eight of 51 participants had an FCRI-SF score above the threshold for clinical fear of cancer recurrence. Conclusions and Relevance: This qualitative and survey-based study found that despite having an excellent prognosis, some survivors of localized melanoma, even those who had stage 0, have high rates of fear of cancer recurrence and intense survivorship experiences that affect their psychological well-being.

Topical trametinib for epidermal and sebaceous nevi in a child with Schimmelpenning-Feuerstein-Mims syndrome.

We present the case of a 20-month-old girl with Schimmelpenning-Feuerstein-Mims (SFM) syndrome with extensive head, neck, and torso skin involvement successfully managed with topical trametinib. Trametinib interferes downstream of KRAS and HRAS in the MAPK signaling pathway, of which KRAS was implicated in our child's pathogenic variant. Although other dermatologic conditions have shown benefit from oral trametinib, its topical use has not been well reported. Our patient showed benefit from the use of twice-daily topical trametinib, applied to the epidermal and sebaceous nevi over a 16-month period, leading to decreased pruritus and thinning of the plaques.

Virtual reality and noise canceling headphone distraction during pediatric dermatologic procedures.

Pain and anxiety related to medical procedures have long been recognized as a significant healthcare concern. If a patient's procedural pain and anxiety are not addressed, long-term physical and psychological sequelae including increased perceived pain, anxiety, disruptive behavior, trauma reactions, or refusal of future procedures can occur. The objective of our study was to assess the utility of a virtual reality (VR) headset or noise-canceling headphones (HP) compared to treatment as usual (TAU) in reducing pain and anxiety during pediatric dermatology procedures. Results indicated a significant difference between pre- versus post-procedure anxiety in the VR and HP groups but not the TAU group suggesting non-pharmacologic technology-based interventions such as VR and headphones may reduce patients' anxiety during pediatric dermatology procedures.

Psychosocial considerations in the management of vitiligo.

Vitiligo is a depigmenting skin disease that can carry significant psychosocial impact. Health care providers play a crucial role in shaping patients' understanding of their condition, approach to treatment, and coping mechanisms. In this contribution, we review the psychosocial aspects of vitiligo management, including the debate over the "disease-ification" of vitiligo, its effects on quality of life and mental health, and methods to holistically assist affected individuals beyond just treatment of the vitiligo itself.

Ichthyosis, petechiae, and arthrogryposis in a neonate.

Gaucher disease is a rare lysosomal storage disorder caused by a deficiency in glucocerebrosidase. This enzyme deficiency leads to the accumulation of toxic metabolites in various organs. Multiple subtypes of this disease have been described; however, the perinatal-lethal form is extremely rare and challenging to diagnose. We present a case of a newborn girl with ichthyosis, petechiae, and arthrogryposis, later found to be homozygous for a pathogenic variant of the glucocerebrosidase gene. This case highlights the potential role of dermatologists in the recognition of this rare disease.

Gastrostomy tube feeding in epidermolysis bullosa: A multi-center assessment of caregiver satisfaction.

BACKGROUND/OBJECTIVES: Gastrostomy tubes (G-tubes) are a feasible intervention for nutritional support in patients with epidermolysis bullosa (EB) who suffer from gastrointestinal involvement. However, some parents may be apprehensive about this surgical intervention. The purpose of this study is to assess caregiver satisfaction and experiences after G-tube insertion in children with EB. METHODS: Nine institutions sent an anonymous 89-item online questionnaire to caregivers of patients with EB and active G-tubes during an 8-week study period. The questionnaire consisted of eight sections and was formatted with multiple choice, Likert scales, dichotomous scales (yes/no), and free text. RESULTS: Twenty caregiver surveys were completed. All caregivers were parents (100%). The average age of G-tube placement was 2.7 years (SD: 2.1, range 0-8) and duration of the G-tube utilization was an average of 12.0 years (SD: 7.8, range 1-25). EB subtypes were dystrophic (n = 15/20, 75%), junctional (n = 3/20, 15%), and simplex (n = 2/10, 10%). Almost all caregivers (n = 18/20, 90%) reported high levels of satisfaction with G-tube management (scores > 20 on the modified SAGA-8 scale). More than half of caregivers (n = 12/20, 60%) reported that, retrospectively, they would have desired earlier adoption of the G-tube. Caregivers rated mealtimes as more enjoyable and easier both for themselves and the individual with EB post-G-tube placement. CONCLUSION: The survey demonstrates a high rate of satisfaction for G-tube use among caregivers of patients with EB across varying ages and household settings. The free-text responses provide unique insight into caregiver's individual experiences regarding G-tubes to be considered for future studies.

A Case of a Rare Histologic Variant: Cavernous Hemangioma-Like Kaposi Sarcoma.

ABSTRACT: Kaposi sarcoma (KS) is an endothelial tumor associated with human herpesvirus 8. Cutaneous lesions can present with pink or purple patches, plaques, and nodules which can be ulcerated. The main subtypes of KS generally have similar histologic appearances, with spindle cells and expression of human herpesvirus 8 being characteristic features. However, various histologic variants have been reported. We present the case of a 55-year-old man with cutaneous KS with cavernous hemangioma-like histological features. Cavernous hemangioma-like KS is a rare morphologic type of KS, with only a handful of cases reported in the literature.

Cutaneous collagenous vasculopathy in a pediatric patient.

We report the case of a 13-year-old female who presented with punctate, erythematous macules coalescing into patches on the upper extremities and left thigh. A skin biopsy demonstrated dilated capillary-sized blood vessels in the papillary dermis consistent with a diagnosis of cutaneous collagenous vasculopathy (CCV). To our knowledge, this is the youngest patient to present with CCV and will represent the third pediatric case in the literature.

Blood Glucose Testing Lancet and Paper Clip as a Milia Extractor.

In low-resource settings, dermatologists may not have the preferred tools needed to evaluate a patient or perform a procedure. Commonplace affordable supplies can be substituted when needed. We describe the use of a blood glucose testing lancet and a paper clip for milia extraction.

A case of multiple, bilateral Becker's Nevi of the trunk and lower extremities in a young girl: A case report.

A Becker's nevus typically appears as a single unilateral, well-demarcated, hyperpigmented patch over the upper trunk during adolescence. It uncommonly presents as multiple and bilateral patches and rarely involves a lower extremity. We describe the unusual case of a child with multiple, bilateral Becker's nevi of the trunk and lower extremities present since birth.

Reducing anxiety and preventing trauma in pediatric epidermolysis bullosa.

Although dermatologists are well-trained in the medical management of complex skin disease, psychosocial care often exceeds a dermatologist's skillset. We aim to elucidate major factors to consider in the comprehensive management of pediatric epidermolysis bullosa (EB) and provide care recommendations. There are many types of trauma a child with EB may experience, from social to psychological to medical. We include information on trauma-informed care and advice for the dermatologist and multidisciplinary team regarding patient-centered and family-centered approaches to recognizing and reducing anxiety and trauma in EB patients.

A case of prolidase deficiency in a male patient.

Prolidase deficiency is an extremely rare, autosomal recessive disorder resulting in defective collagen formation. We report a case of prolidase deficiency in a male child, highlighting the dermatologic features. Early diagnosis is important as these patients encounter significant multisystem comorbidities requiring multispecialty care.