Vomiting and Dehydration in a 2-Year-Old.

A 2-year-old girl with a past medical history of cutaneous mastocytosis and eczema presented with 1 day of yellow-green, nonbloody vomiting, bradycardia, and listlessness. She was evaluated by her pediatrician and sent to the emergency department because of concern for dehydration. In the emergency department, she improved with fluid rehydration but still had decreased energy and bradycardia. Her electrocardiogram revealed sinus bradycardia, and laboratory results did not reveal any electrolyte abnormalities. Glucose levels were normal. An abdominal radiograph revealed a moderate-to-large stool burden, and the results of a computed tomography scan of the head were normal. An abdominal ultrasound was obtained to evaluate for intussusception. The ultrasound revealed a blind-ending tubular structure in the right-lower quadrant with adjacent free fluid, which was concerning for appendicitis. The patient was admitted to the surgical service for further management and was taken to the operating room, where a definitive diagnosis was made.

Myths on Chemical Burns in the Diaper Area.

Over the past several years, a number of articles and online posts have circulated on the Internet associating use of disposable and cloth diapers with chemical burns on babies' skin. Because both mild chemical burns and diaper dermatitis (diaper rash) can cause skin redness and peeling, it is not surprising that some confusion has arisen regarding the association between these two conditions. However, diapers cannot cause chemical burns because they are made of inert materials. Diaper rash and chemical burns are distinct conditions that require different evaluation and treatment, which is why it is important for pediatricians to help parents understand the difference.

From the Medical Board of the National Psoriasis Foundation: Treatment targets for plaque psoriasis.

BACKGROUND: An urgent need exists in the United States to establish treatment goals in psoriasis. OBJECTIVE: We aim to establish defined treatment targets toward which clinicians and patients with psoriasis can strive to inform treatment decisions, reduce disease burden, and improve outcomes in practice. METHODS: The National Psoriasis Foundation conducted a consensus-building study among psoriasis experts using the Delphi method. The process consisted of: (1) literature review, (2) pre-Delphi question selection and input from general dermatologists and patients, and (3) 4 Delphi rounds. RESULTS: A total of 25 psoriasis experts participated in the Delphi process. The most preferred instrument was body surface area (BSA). The most preferred time for evaluating patient response after starting new therapies was at 3 months. The acceptable response at 3 months postinitiation was either BSA 3% or less or BSA improvement 75% or more from baseline. The target response at 3 months postinitiation was BSA 1% or less. During the maintenance period, evaluation every 6 months was most preferred. The target response at every 6 months maintenance evaluation is BSA 1% or less. LIMITATIONS: Although BSA is feasible in practice, it does not encompass health-related quality of life, costs, and risks of side effects. CONCLUSION: With defined treatment targets, clinicians and patients can regularly evaluate treatment responses and perform benefit-risk assessments of therapeutic options individualized to the patient.

Reduced H3K27me3 Expression Is Common in Nodular Melanomas of Childhood Associated With Congenital Melanocytic Nevi But Not in Proliferative Nodules.

The formation of a nodule within a congenital melanocytic nevus (CMN) raises concerns about possible melanoma. Most new nodular growths that develop during childhood, however, are benign proliferative nodules (PN); melanoma is very rare. The distinction of melanoma from PN can at times be difficult clinically and histopathologically, requiring ancillary molecular tests for diagnosis. Although the application of molecular methods has revealed new insights into the mutational and genomic landscape of childhood melanomas, little is known about epigenetic events that may drive the growth of a melanoma or PN in a CMN. In this study we compared the expression of H3K27me3, a key regulator in chromatin remodelling-controlled transcription, in PNs and pediatric nodular melanomas arising within medium-sized to large CMN by immunohistochemistry. Significant loss of H3K27me3 expression was seen in 4 of 5 melanomas, but not in any of the 20 PNs. This observation suggests that epigenetic events likely play a role in the pathogenesis of melanoma developing in the dermis or subcutis of CMN. Furthermore, assessing for H3K27me3 expression by immunohistochemistry may be diagnostically useful for problematic cases.

Prepubertal Melanoma Arising within a Medium-Sized Congenital Melanocytic Nevus.

We report the case of an 8-year-old child who developed a 9.4-mm-deep melanoma within a medium-sized congenital melanocytic nevus on the scalp. Genetic analysis revealed an activating NRAS Q61R mutation within the melanoma, which is more commonly associated with large or giant congenital melanocytic nevi. This case demonstrates that even a "low-risk" congenital melanocytic nevus at a "low-risk" age must be monitored regularly for the development of malignancy.

Beyond Psoriasis: Novel Uses for Biologic Response Modifiers in Pediatric Dermatology.

Dermatologists have witnessed the increasing availability of novel biologic response modifiers for the treatment of inflammatory and autoimmune diseases in recent years. The most common dermatologic indication for the use of biologic response modifiers in adults is psoriasis, but the U.S. Food and Drug Administration has not approved any of these agents for use in any dermatologic disease in children with the exception of omalizumab, and as such, use in this population is considered off-label. In this review, we focus on the use of these agents in children to treat inflammatory skin diseases other than psoriasis, including atopic dermatitis, hidradenitis suppurativa, pemphigus vulgaris, bullous pemphigoid, and toxic epidermal necrolysis, with an emphasis on the use of etanercept, infliximab, rituximab, omalizumab, and ustekinumab. By highlighting novel uses of these agents, particularly for the treatment of dermatologic conditions for which optimal therapies are yet to be established, we hope to raise awareness of the potential use of this class of medications to treat inflammatory skin diseases in children.

Obesity and the metabolic syndrome in pediatric psoriasis.

Psoriasis is a common, chronic inflammatory dermatosis that often has its onset during childhood. There is increasing evidence that psoriasis in adults is associated with obesity, the metabolic syndrome, and associated comorbidities, including insulin resistance/type 2 diabetes, dyslipidemia, hypertension, and cardiovascular disease. This association is postulated to arise, at least in part, as a result of a systemic proinflammatory state that is mediated by adipose tissue. Several recent observational studies suggest that children and adolescents with psoriasis may be at increased risk of being overweight and obese as well as having an increased risk for features of the metabolic syndrome. Such an association raises concern with regards to the long-term health implications for children and adolescents with psoriasis and suggests that better awareness, evaluation, and management of overweight and obese patients and associated metabolic disease are warranted in this population.

Biologic response modifiers and pediatric psoriasis.

The efficacy and safety of biologic response modifiers such as etanercept, adalimumab, infliximab, and ustekinumab have been demonstrated in the treatment of psoriasis in adults, but none are currently approved for the treatment of psoriasis in children in the United States, and only etanercept is approved for the treatment of psoriasis in children in the European Union. Through case reports, case series, and a large clinical trial of the use of etanercept, the literature supports the use of these agents to treat psoriasis in children. Data on the use of the tumor necrosis factor-α antagonists etanercept, adalimumab, and infliximab in the treatment of other inflammatory diseases in children-namely Crohn's disease, juvenile arthritis, and uveitis--support their safety profile in children.

Psoriasis in childhood: effective strategies to improve treatment adherence.

Psoriasis is a relatively common chronic inflammatory skin disease in children for which there is no cure. Most children have mild disease that can be managed with topical therapy as opposed to phototherapy or systemic therapy. Despite the mild presentation of psoriasis in most children, the disease can have a significant impact on quality of life due to the need for ongoing treatment, the frequently visible nature of the cutaneous manifestations, and the social stigma that is associated with psoriasis. Adherence to treatment, in particular topical therapy, is often poor in adults and compromises response to therapy and medical provider management strategies. Multiple factors that may contribute to nonadherence in adults with psoriasis have been identified, including lack of education on the disease and expectations for management, issues related to ease of use and acceptability of topical medications, and anxiety regarding possible medication side effects. There is currently no published data on adherence in the pediatric psoriasis population; however, poor adherence is often suspected when patients fail to respond to appropriate therapy. General strategies used to assess adherence in other pediatric disease populations can be applied to children with psoriasis, and interventions that reflect experience in other chronic dermatologic disorders such as atopic dermatitis may also be helpful for medical providers caring for children with psoriasis.

Combined immune deficiency in a patient with a novel NFKB2 mutation.

NFKB2 encodes the p100/p52 protein, a critical mediator of the canonical and noncanonical NFkB signaling pathways. Here we report the comprehensive immune evaluation of a child with a novel NFKB2 mutation and provide evidence that aberrant NFKB2 signaling not only causes humoral immune deficiency, but also interferes with the TCR-mediated proliferation of T cells. These observations expand the known phenotype associated with NFKB2 mutations.

Retrospective analysis of beta-blocker instituted for treatment of hemangiomas (RABBIT study).

OBJECTIVE: To evaluate the safety and efficacy of our institutional beta-blocker protocol for treatment of complicated infantile hemangiomas (IH). STUDY DESIGN: A retrospective descriptive study of 76 infants/children with IH treated with oral propranolol at the Children's Hospital of Philadelphia between June 2008 and August 2010 was performed, assessing both the safety and efficacy of propranolol. Based on preliminary data showing hemangioma recrudescence off-treatment, we reviewed 9 additional patients with recrudescence between August 2010 and December 2011. RESULTS: Mild adverse events included asymptomatic bradycardia, gastrointestinal symptoms, asymptomatic hypotension, cool hands/feet, asymptomatic hypoglycemia, and sleep disturbance. Sixteen patients had recrudescence of IH off-treatment, with propranolol discontinued at a median age of 14 months (interquartile range 10-15 months). CONCLUSIONS: Propranolol appears to be associated with minor, not severe symptomatic adverse events. Propranolol appears to be effective in treating complicated IH. Recrudescence can occur off-treatment, even with discontinuing propranolol as late as 15 months of age.

Dressings and Products in Pediatric Wound Care.

Significance: The increasing complexity of medical and surgical care provided to pediatric patients has resulted in a population at significant risk for complications such as pressure ulcers, nonhealing surgical wounds, and moisture-associated skin damage. Wound care practices for neonatal and pediatric patients, including the choice of specific dressings or other wound care products, are currently based on a combination of provider experience and preference and a small number of published clinical guidelines based on expert opinion; rigorous evidence-based clinical guidelines for wound management in these populations is lacking. Recent Advances: Advances in the understanding of the pathophysiology of wound healing have contributed to an ever-increasing number of specialized wound care products, most of which are predominantly marketed to adult patients and that have not been evaluated for safety and efficacy in the neonatal and pediatric populations. This review aims to discuss the available data on the use of both more traditional wound care products and newer wound care technologies in these populations, including medical-grade honey, nanocrystalline silver, and soft silicone-based adhesive technology. Critical Issues: Evidence-based wound care practices and demonstration of the safety, efficacy, and appropriate utilization of available wound care dressings and products in the neonatal and pediatric populations should be established to address specific concerns regarding wound management in these populations. Future Directions: The creation and implementation of evidence-based guidelines for the treatment of common wounds in the neonatal and pediatric populations is essential. In addition to an evaluation of currently marketed wound care dressings and products used in the adult population, newer wound care technologies should also be evaluated for use in neonates and children. In addition, further investigation of the specific pathophysiology of wound healing in neonates and children is indicated to promote the development of wound care dressings and products with specific applications in these populations.

Diagnosis and treatment of pediatric psoriasis: current and future.

Psoriasis is a common yet complex inflammatory dermatosis that may be seen in infants, children, and adolescents. The clinical presentation and course may be quite variable, and while patients with mild disease are often easily managed, those with recalcitrant or more severe disease often present a therapeutic dilemma given the number of therapies available and the relative lack of data on the efficacy and safety of use of these therapies in children. This review presents the reader with an overview of the current understanding of the pathophysiology, diagnosis, and treatment of pediatric psoriasis, with an emphasis on the available data in the literature that pertains to the use in children of currently available topical and systemic therapies, including topical corticosteroids, vitamin D analogs, phototherapy, systemic immunosuppressive medications, and biologic agents.