Topical rapamycin for acanthosis nigricans in the Fitzpatrick IV/V adolescent population.

Dermatologically, FGFR3 mutations can lead to acanthosis nigricans (AN), epidermal nevi, and seborrheic keratosis. A recent case report found that topical rapamycin (sirolimus) can improve FGFR3-induced epidermal nevi with AN features in children, specifically with Fitzpatrick skin type (FST) I/II, and we would like to expand these findings to skin plaques with extensive AN-like features in the FST IV/V adolescent population. An 18-year-old female with FST IV/V and FGFR3-induced hypochondroplasia presented to our clinic with extensive AN-like plaques. Significant improvement with lightening and thinning of the plaques was observed after applying 1% topical rapamycin cream twice daily. Topical rapamycin should be considered as a treatment option for AN, particularly in FST IV/V adolescents with FGFR3-induced AN.

Effectiveness of Early Laser Treatment in Surgical Scar Minimization: A Systematic Review and Meta-analysis.

BACKGROUND: Studies investigating the efficacy of lasers to minimize early surgical scars are low powered and report variable results. To further examine the evidence, the authors performed a systemic review and meta-analysis. OBJECTIVE: To present the evidence of randomized controlled trials investigating the efficacy of laser modalities in minimizing surgical scars when applied <1 month after operation. MATERIALS AND METHODS: A literature search of PubMed, EMBASE, Northern Light Life Sciences Conference Abstracts, and Cochrane Library was performed between November 6, 2015, and November 20, 2015. After assessing for inclusion, data extraction used the PRISMA checklist. Assessment for quality, validity, and risk of bias applied a scale devised by Jadad and colleagues, the Oxford Pain Validity Scale, and the RevMan risk of bias assessment tool, respectively. The GRADEpro application graded overall quality, and statistical analysis was performed with RevMan. RESULTS: Approximately 4,373/4,397 abstracts and 16/24 full articles were excluded using predefined criteria, leaving 8 articles in the systematic review and 4 in the meta-analysis. The primary outcome reached statistical significance favoring the intervention group with standardized mean difference 0.39 (95% confidence interval, 0.05-0.74) and p = .03. CONCLUSION: The outcome supports the efficacy of lasers in minimizing primarily closed surgical scars when treated <1 month after surgery.

Treatment of benign cephalic histiocytosis with topical 1% rapamycin ointment.

We report a pediatric case of extensive, progressive benign cephalic histiocytosis (BCH) involving the face, trunk, and extremities with response of facial lesions to treatment with topical 1% rapamycin. A split-face model was used to demonstrate improvement on the treated side versus the untreated side. After physician and parental perception of effectiveness, based in part on photodocumentation, subsequently both cheeks were treated with continued improvement.

Topical sirolimus for treatment of a venolymphatic malformation in an adolescent girl.

Sirolimus has emerged as a promising treatment for complex congenital vascular malformations but is limited because of systemic side effects. Topically compounded formulations, which have been found to be successful in the treatment of several other conditions, are a preferred drug delivery system to provide local therapy. We report a case of a venolymphatic malformation in an adolescent girl treated with topical 1% sirolimus cream leading to improvement in appearance and associated symptoms.

Warming up to the idea of wet wraps.

Atopic dermatitis (AD) is a prevalent condition in the pediatric population that can have a significant effect on a child's quality of life. Management is multifactorial, involving topical pharmacotherapy, emollients, and a bathing regimen in conjunction with close supervision from the caregiver and physician. In the case of moderate to severe or refractory AD, wet wraps can be used. As part of our wet wrap regimen, we propose warming damp cotton pajamas in the dryer before application. This makes the wet wraps more comfortable for children, increasing adherence to the prescribed regimen.

Multilineage somatic activating mutations in HRAS and NRAS cause mosaic cutaneous and skeletal lesions, elevated FGF23 and hypophosphatemia.

Pathologically elevated serum levels of fibroblast growth factor-23 (FGF23), a bone-derived hormone that regulates phosphorus homeostasis, result in renal phosphate wasting and lead to rickets or osteomalacia. Rarely, elevated serum FGF23 levels are found in association with mosaic cutaneous disorders that affect large proportions of the skin and appear in patterns corresponding to the migration of ectodermal progenitors. The cause and source of elevated serum FGF23 is unknown. In those conditions, such as epidermal and large congenital melanocytic nevi, skin lesions are variably associated with other abnormalities in the eye, brain and vasculature. The wide distribution of involved tissues and the appearance of multiple segmental skin and bone lesions suggest that these conditions result from early embryonic somatic mutations. We report five such cases with elevated serum FGF23 and bone lesions, four with large epidermal nevi and one with a giant congenital melanocytic nevus. Exome sequencing of blood and affected skin tissue identified somatic activating mutations of HRAS or NRAS in each case without recurrent secondary mutation, and we further found that the same mutation is present in dysplastic bone. Our finding of somatic activating RAS mutation in bone, the endogenous source of FGF23, provides the first evidence that elevated serum FGF23 levels, hypophosphatemia and osteomalacia are associated with pathologic Ras activation and may provide insight in the heretofore limited understanding of the regulation of FGF23.

Characteristics and treatment pathways in pediatric and adult hidradenitis suppurativa: An examination using real world data.

Background: Hidradenitis suppurativa (HS) is a chronic, debilitating, inflammatory disease. Contemporaneous real-world data can be used to elucidate the clinical treatment of pediatric patients and how treatment strategies compare with adult hidradenitis suppurativa patients. Objective: The objective of this study is to evaluate clinical and treatment characteristics of pediatric and adult HS patients. Methods: HS adult and pediatric patients were identified in 3 the United States administrative claims databases during the study period between 2016 to 2021. Patients were required to have 2 diagnostic codes for HS and have at least 365 days of prior observation time to the first HS diagnosis. Results: Pediatric and adult HS treatments were similar. The proportions of subjects treated with topical and oral antibiotic or oral antibiotic alone or topical medication alone or surgery alone covered 90% of the treated pediatric subjects and 91% of treated adult subjects. The remaining proportion of subjects received other treatment combinations. Limitations: The databases represent subjects with commercial or government insurance coverage and thus do not necessarily represent the broader US population. The databases do not capture information about medications obtained without insurance. Conclusions: Although subtle differences exist, this study confirms that topical and systemic therapeutic treatment of HS in adults and adolescents is very similar.

Intrauterine Herpes Simplex Virus Infection Presenting as a Zosteriform Eruption in a Newborn.

There is significant morbidity and mortality associated with the transmission of herpes simplex virus (HSV) from pregnant women to their fetus or newborn. Although most commonly transmitted in the peripartum period, in rare cases HSV can lead to intrauterine infection. Cutaneous lesions are the most common manifestation of intrauterine HSV, and have a wide spectrum of presentation. We present a rare case of intrauterine HSV-2 infection presenting with a zosteriform eruption mimicking congenital varicella syndrome in a newborn.

Psoriatic plaques "koebnerizing" to areas of acanthosis nigricans in an obese female: clues to a common pathway?

Recent evidence suggests that the activation of several growth factor receptors (EGFR, IGFR1, and FGFRs) is a possible cause of acanthosis nigricans, a skin disorder characterized by velvety thin plaques in skin folds and often seen in patients with insulin resistance. The authors report a 14-year-old obese (body mass index = 38.5kg/m(2)) girl with a history of polycystic ovarian syndrome and pre-diabetes who presented with psoriatic plaques in her scalp and, subsequently, in areas mostly confined to where she had characteristic lesions of acanthosis nigricans. The authors propose that this as-of-yet unreported observation may represent a preferential koebnerization phenomenon where the abnormal keratinocyte proliferation in acanthosis nigricans may serve as the epidermal "micro-trauma" necessary to incite the prototypical isomorphic response seen in psoriasis.

The Eraser Challenge Among School-age Children.

Skin-related, self-destructive behaviors are being increasingly reported among school-age children. Often disguised as innocuous "challenges," these disturbing actions may have the potential for serious and permanent sequelae. Interest and subsequent participation in these behaviors may also be spread "virally," facilitated by social networking sites that allow participants-regardless of age-to share photographs or videos of the activities in question. Consequently, parents and health care providers must learn to recognize signs and symptoms of these disturbing behaviors and make concerted efforts to protect this uniquely vulnerable population of pediatric patients.