Stigmatization and Mental Health Impact of Chronic Pediatric Skin Disorders.

Importance: Chronic skin disorders in children frequently are visible and can cause stigmatization. However, the extent of stigmatization from chronic skin disease and association with mental health needs further study.

Objective: To examine the extent of stigma, dependence on disease visibility and severity, and association with mental health and quality of life (QOL) in chronic pediatric skin disease.

Dermatology Life Quality Index survey in patients with Down syndrome and caregivers.

Down syndrome (DS) is associated with many dermatological conditions, including hidradenitis suppurativa, folliculitis, and alopecia areata. Despite the high incidence of skin conditions in this population, there are no quality of life (QoL) studies in the dermatology literature focused on patients with DS or their caregivers. The frequently used QoL assessment tool, the Dermatology Life Quality Index (DLQI), has yet to be studied in this population.

Multicenter Study of Long-Term Outcomes and Quality of Life in PHACE Syndrome after Age 10.

Objective: To characterize long-term outcomes of PHACE syndrome.

Study Design: Multicenter study with cross-sectional interviews and chart review of individuals with definite PHACE syndrome ≥10 years of age. Data from charts were collected across multiple PHACE-related topics.

Intralesional measles, mumps, and rubella vaccine after failure of intralesional Candida antigen for the treatment of recalcitrant pediatric warts.

Numerous studies have investigated the efficacy of intralesional immunotherapy for warts, but there are a lack of studies investigating the efficacy of alternative intralesional immunotherapies following failure of initial intralesional immunotherapy. In this retrospective study, we aimed to investigate the efficacy of intralesional measles, mumps, and rubella vaccine for the treatment of pediatric warts following failure of intralesional therapy with Candida antigen. Following intralesional measles, mumps, and rubella vaccine administration, 8/51 (15.

A Rare Case of Acne Medication-Induced Drug Reaction With Eosinophilia and Systemic Symptoms.

Introduction: Acne vulgaris is the most common skin condition in late adolescence and frequently requires systemic treatment with antibiotics or androgen receptor blockers in moderateto- severe cases.

Case Presentation: We report the case of a 17-year-old adolescent female with new onset fever, headache, and pruritic rash 1 month after she started doxycycline and spironolactone for the treatment of acne vulgaris. Later, she developed eosinophilia and transaminitis.

A Comparison of Psoriasis Severity in Pediatric Patients Treated With Methotrexate vs Biologic Agents.

Importance: Few studies have compared the use of methotrexate and biologics, the most commonly used systemic medications for treatment of moderate to severe psoriasis in children.

Objective: To assess the real-world, 6-month reduction in psoriasis severity and long-term drug survival (rate and duration of adherence to a specific drug) of methotrexate vs biologics in plaque psoriasis in children.

Design, Setting, And Participants: A retrospective medical records review was conducted at 20 European and North American centers.

Second-Hit, Postzygotic PMVK and MVD Mutations in Linear Porokeratosis.

Importance: Linear porokeratosis features linear and whorled configurations of keratotic papules and plaques, with coronoid lamellae present on histologic examination. Because linear porokeratosis manifests in the lines of Blaschko representing the dorsoventral migration patterns of keratinocyte precursors, it has been suggested that postzygotic somatic mutation underlies the disease. However, no genetic evidence has supported this hypothesis to date.

Localized infantile hemangiomas of the face and scalp: Predilection for the midline and periorbital and perioral skin.

Background/objectives: Infantile hemangiomas are common vascular tumors. Identifying sites of predilection may provide insight into pathogenesis. Previous studies have suggested a predilection for the boundary of facial metameres.

CARD14-associated papulosquamous eruption: A spectrum including features of psoriasis and pityriasis rubra pilaris.

Background: Heterozygous mutations in caspase recruitment domain family member 14 gene (CARD14) have been shown to be associated with psoriasis and familial pityriasis rubra pilaris (PRP). Many subjects with CARD14 mutations display features of both disorders, which can result in diagnostic uncertainty. In addition, these eruptions are often recalcitrant to conventional psoriasis therapies such as methotrexate, oral retinoids, and tumor necrosis factor-α inhibitors.

Characteristics of pediatric recurrent erythema multiforme.

Background: Erythema multiforme (EM) is an acute condition characterized by distinctive target lesions of the skin often accompanied by mucosal ulcers. A subset of individuals experience frequent episodes of recurrent EM, which is rare and poorly understood, especially in children.

Objective: To characterize clinical features, laboratory findings, and treatment responses of pediatric recurrent EM.

Analyzing the Genetic Spectrum of Vascular Anomalies with Overgrowth via Cancer Genomics.

Vascular anomalies are variably associated with overgrowth, skeletal anomalies, and abnormalities of the brain, leptomeninges, and eye. We assembled a 16-institution network to determine the range of genetic variants associated with a spectrum of vascular anomalies with overgrowth, ranging from mild to severe. Because of the overlap between cancer-associated variants and previously described somatic variants in vascular overgrowth syndromes, we employed tumor genetic profiling via high-depth next-generation sequencing using a panel to assay affected tissue from a diverse cohort of subjects with vascular anomalies with overgrowth.

The spectrum of manifestations in desmoplakin gene (DSP) spectrin repeat 6 domain mutations: Immunophenotyping and response to ustekinumab.

Background: The immune abnormalities underlying the ichthyoses are poorly understood.

Objective: To determine the immunophenotype of an ichthyosis resulting from mutations in the spectrin repeat 6 (SR6) domain of desmoplakin gene (DSP) and target therapy on the basis of molecular pathogenesis.

Methods: Immunophenotyping was performed by using the blood and skin of a girl with SR6 region DSP mutations causing erythroderma/ichthyosis and cardiomyopathy.

Safety of Systemic Agents for the Treatment of Pediatric Psoriasis.

Importance: Use of systemic therapies for moderate to severe psoriasis in children is increasing, but comparative data on their use and toxicities are limited.

Objective: To assess patterns of use and relative risks of systemic agents for moderate to severe psoriasis in children.

Design, Setting, And Participants: A retrospective review was conducted at 20 centers in North America and Europe, and included all consecutive children with moderate to severe psoriasis who used systemic medications or phototherapy for at least 3 months from December 1, 1990, to September 16, 2014.

Differential Growth of , Which Alters Expression of Virulence Factors, Dominant Antigens, and Surface-Carbohydrate Synthases, Governs the Apparent Virulence of SchuS4 to Immunized Animals.

The gram-negative bacterium () is both a potential biological weapon and a naturally occurring microbe that survives in arthropods, fresh water amoeba, and mammals with distinct phenotypes in various environments. Previously, we used a number of measurements to characterize grown in Brain-Heart Infusion (BHI) broth as (1) more similar to infection-derived bacteria, and (2) slightly more virulent in naïve animals, compared to grown in Mueller Hinton Broth (MHB). In these studies we observed that the free amino acids in MHB repress expression of select virulence factors by an unknown mechanism.

Complete resolution of trichodysplasia spinulosa in a pediatric renal transplant patient: Case report and literature review.

TS of immunosuppression is a rare, disfiguring dermatologic condition caused by TS-associated polyomavirus in immunosuppressed patients. It is difficult to treat, with no clearly described approach to resolve the condition completely and safely. We report a child with a renal transplant who developed TS and was treated with significant reduction in immunosuppression and transient use of cidofovir cream.

- Immune Cell Activator, Suppressor, or Stealthy Evader: The Evolving View from the Petri Dish.

One of the hallmarks of pulmonary tularemia, which results from inhalation of - a significant bioterrorism concern, is the lack of an acute T1-biased inflammatory response in the early phase of disease (days 1-3) despite significant bacterial loads. In an effort to understand this apparent hypo-responsiveness, many laboratories have utilized cell-based models as tools to probe the nature and consequences of host cell interactions with . The first uses of this model suggested that mammalian host cells recognize this bacterium principally through TLR2 to evoke a robust, classical T1-biased cytokine response including TNF, IL-6, IL-1β, and IFN-γ.

Topical Timolol Maleate Treatment of Infantile Hemangiomas.

Background: There has been a dramatic increase in the off-label use of ophthalmic timolol maleate, a β-blocker used for infantile hemangioma (IH) treatment as a topical counterpart to oral propranolol. Its safety and efficacy in a pediatric population with IH have not been evaluated in a large cohort. Our goal was to retrospectively assess timolol's effectiveness, discern characteristics associated with response, and document reported adverse events.

Adverse Events in Young and Preterm Infants Receiving Topical Timolol for Infantile Hemangioma.

Background: The success of oral propranolol for treatment of infantile hemangiomas (IHs) has led practitioners to use topical β-blockers. In preterm infants, clinicians frequently turn to topical timolol, with the presumption that topical application will result in less systemic absorption. We used Holter monitoring to assess for drug-induced bradycardia in high-risk infants.