A review of hair removal modalities in pediatric patients: Ethical and clinical considerations.

Unwanted hair is a common concern among patients presenting to pediatric dermatology clinics, and parents and patients alike inquire about the safety of methods employed for elective removal. Various methods of hair removal exist with different levels of invasiveness and permanence, from simple mechanical depilation to light-based therapies. All methods of hair removal appear to be safe and generally well tolerated in children, and there are no age restrictions to any modality. In this review, we aim to address the available literature on the safety and efficacy of hair removal modalities in pediatric patients and propose guidance on how to manage requests for at-home and in-office therapies within an ethical framework.

Ulcerated diaper dermatitis: Child abuse mimicker.

Irritant diaper dermatitis is a common dermatologic problem among infants and young children. Severe erosive presentations, although uncommon, are diagnostically challenging and can mimic non-accidental trauma (NAT). Diagnosing inflicted injury and NAT where it does not exist can cause parental distress, yet failing to diagnose inflicted injury and NAT can result in re-injury. We describe three cases of severe erosive diaper dermatitis in pediatric patients aged 2-6 years that were initially concerning for inflicted scald burn or neglect.

Severe reactive infectious mucocutaneous eruption mimicking drug-induced epidermal necrolysis triggered by norovirus.

Reactive infectious mucocutaneous eruption (RIME) is an eruptive mucositis with varying degrees of cutaneous involvement presumed to be due to an immunologic response to various infectious pathogens. Most reported cases occur after a prodromal upper respiratory illness. We present a patient with a particularly severe case mimicking drug-induced epidermal necrolysis found to be triggered by asymptomatic norovirus infection, a virus not previously reported in association with RIME.

Characteristics and complications of anogenital infantile hemangiomas: A multicenter retrospective analysis.

BACKGROUND: Infantile hemangiomas (IHs) of the anogenital region remain poorly characterized. OBJECTIVE: To examine the distribution, ulceration rate, and associated congenital anomalies of anogenital IHs. METHODS: Retrospective study at 8 tertiary referral centers. RESULTS: A total of 435 infants with an IH of the anogenital region were enrolled (of which, 319 [73%] were girls). Congenital anomalies were present in 6.4% (n = 28) of infants with an anogenital IH. Segmental or partial segmental anogenital IHs ulcerated in 72% (n = 99 of 138) of infants, whereas 45% (n = 133 of 297) of focal anogenital IHs experienced ulceration (P < .001). In a multivariable logistic regression analysis, segmental or partial segmental morphology (adjusted odds ratio [aOR], 2.70; 95% CI, 1.60-4.64), mixed type (aOR, 3.44; 95% CI, 2.01-6.07), and perianal (aOR, 3.01; 95% CI, 1.53-6.12) and buttocks location (aOR, 2.08; 95% CI, 1.17-3.76) had increased odds of ulceration. Segmental or partial segmental IHs of the genitalia were confined to distinct anatomic territories and were predominantly distributed unilaterally, with a linear demarcation at the perineal raphe. LIMITATIONS: Possible selection bias, given recruitment at tertiary referral centers. CONCLUSION: This study improves our understanding of high-risk features of anogenital IHs and demonstrates that genital segmental or partial segmental IHs develop within distinct anatomic territories.

The utility of laboratory testing for pediatric patients undergoing isotretinoin treatment.

Isotretinoin, the most effective treatment for severe cystic acne, involves laboratory monitoring. In this retrospective case series of 130 pediatric patients taking isotretinoin, there were significant increases in cholesterol (143.9 mg/dl to 155.3 mg/dl), triglycerides (81.8 mg/dl to 115.2 mg/dl), and low-density lipoprotein (82.0 mg/dl to 98.1 mg/dl), and a decrease in high-density lipoprotein (50.0 mg/dl to 44.7 mg/dl) from baseline to follow-up (p < .05); there were no significant changes in liver enzymes. None of the patients had clinical sequelae (triglyceride-induced pancreatitis, retinoid-induced hepatotoxicity) related to their abnormal lab values. These findings question the utility of laboratory monitoring for prevention of severe clinical sequelae in pediatric patients, and suggest testing based on individualized risk factors may be more appropriate.

Feeling the burn: Sunburn recall in staphylococcal scalded skin syndrome.

Staphylococcal scalded skin syndrome (SSSS) occurs primarily in infants and young children due to hematogenous dissemination of Staphylococcus aureus (S. aureus) exfoliative toxin resulting in painful erythema and superficial desquamation of the skin. Herein, we discuss a 7-year-old patient with SSSS in classic locations who additionally presented with photodistributed erythema without desquamation on the chest, arms, and back in locations of a recently healed sunburn. In this report, we discuss sunburn recall phenomenon as a result of SSSS.

Exploring the costs associated with isotretinoin treatment.

The multistep process to obtain an isotretinoin prescription under the iPLEDGE program is challenging for patients, particularly female patients. This retrospective study evaluates the differences in treatment and costs between male and female patients. While male patients had a higher total cost of treatment than female patients, female patients had a higher treatment cost when medication costs were excluded. Female patients who missed prescription windows had a longer treatment course and incurred significantly higher treatment costs than female patients who did not miss a prescription window. The iPLEDGE program places female patients at a disadvantage of incurring higher treatment costs as a consequence of the prescription window requirement.

Evaluating provider knowledge, perception, and concerns about cannabinoid use in pediatric dermatology.

As the use of medical cannabinoids expands, the value and acceptance in pediatric dermatology is poorly characterized. To better assess the current climate, this survey evaluated pediatric dermatologists' familiarity and knowledge about medical cannabis and their interest to research and use cannabinoids. While the fund of knowledge and acceptance was high, providers voiced concerns about psychoactive effects of cannabis and substance abuse among their pediatric patients.

Ecthyma gangrenosum: The critical role of biofilms and other mechanisms of antibiotic resistance and implications for management.

Ecthyma gangrenosum is a rare cutaneous infection that occurs classically in immunocompromised patients with Pseudomonas aeruginosa bacteremia and is associated with a high mortality rate. Causative pathogens may exhibit various antibiotic evasion mechanisms, and thus, treatment may be challenging. We present a case of ecthyma gangrenosum in association with an implantable port in which cultures confirmed ten unique strains of Pseudomonas aeruginosa, highlighting the ability of this pathogen to form biofilms, rapidly mutate and ultimately evade antibiotic therapy. Dermatologists play a key role in the prompt diagnosis of this life-threatening condition, and a thorough understanding of pathogenic mechanisms is critical in selecting an efficacious treatment regimen.

Special management considerations for propranolol use in breastfed infants of mothers taking antihypertensives.

Propranolol is approved for treatment of infantile hemangiomas (IH). IH proliferation coincides with the time when most women are breastfeeding, yet there are no reports describing the clinical effects on infants treated with propranolol while being nursed by mothers on oral antihypertensive medications. We describe two cases of breastfed infants, with mothers taking multiple antihypertensives of different drug classes, who were initiated on propranolol for IH treatment and discuss the theoretical risk of propranolol use in this setting.

Targeted Inhibition of the Epidermal Growth Factor Receptor and Mammalian Target of Rapamycin Signaling Pathways in Olmsted Syndrome.

Importance: Olmsted syndrome is a rare and disabling genodermatosis for which no successful treatment is currently available. Objective: To evaluate the clinical response to the mammalian target of rapamycin (mTOR) inhibitor sirolimus and/or the epidermal growth factor receptor (EGFR) inhibitor erlotinib among patients with Olmsted syndrome. Design, Setting, and Participants: This case series focused on 4 children with treatment-refractory Olmsted syndrome. These children received treatments (initiated in 2017 and 2018) at the outpatient dermatology clinic at the Children's Hospital of Wisconsin in Milwaukee, Wisconsin; Children's National Hospital in Washington, DC; and Hospital Infantil Pequeno Príncipe, Curitiba in Paraná, Brazil. Exposures: Immunohistochemical analyses for mTOR and EGFR activation were performed on skin biopsy specimens from 2 patients. Oral sirolimus was administered to these 2 patients at a dosage of 0.8 mg/m2 twice daily, titrated to a goal trough whole-blood concentration of 10 to 15 ng/mL. Erlotinib was administered to all 4 patients at a dosage of 2 mg/kg/d. Main Outcomes and Measures: Clinical responses were assessed with visual analog scales for pruritus and pain and/or the Children's Dermatology Life Quality Index. Adverse effects were monitored throughout treatment. Results: Four patients (mean [SD] age, 7 [6] years; 2 boys and 2 girls) were analyzed. Lesional skin immunostaining showed increased phosphorylated ribosomal protein S6 (RPS6) and phosphorylated EGFR staining in the epidermis, indicating enhanced mTOR and EGFR signaling activation. Patients 1 and 2 were initially treated with sirolimus, displaying substantial clinical improvement in erythema and periorificial hyperkeratosis afterward. When switched to erlotinib, these patients showed substantial palmoplantar keratoderma (PPK) improvement. Patients 3 and 4 were treated with erlotinib only and later showed rapid and near complete resolution of PPK and substantial improvement in Children's Dermatology Life Quality Index scores. All 4 patients had sustained improvements in pruritus and pain. No severe adverse effects were reported. Conclusions and Relevance: This study's findings suggest that the EGFR-mTOR cascade may play a substantial role in the pathophysiological process of Olmsted syndrome and may serve as a major therapeutic target. Oral sirolimus and erlotinib may be a promising, life-altering treatment for pediatric patients with Olmsted syndrome.

A Pediatric Case of Transformed Mycosis Fungoides in a BRCA2 Positive Patient.

Cutaneous T-cell lymphomas are very rare in children. Although mycosis fungoides is the most common of these rare cutaneous T-cell lymphomas in children, transformation to an aggressive malignancy remains extremely uncommon, and there are no clear guidelines for clinical management in the pediatric population. In addition, the increased usage of next-generation sequencing for pediatric patients with unusual malignancies may result in the discovery of pathogenic germline mutations, though the association between these mutations and the patient's cancer is not always clear. We present here a unique pediatric case of transformed mycosis fungoides in a patient with BRCA2 mutation.

Atypical presentations of congenital hemangiomas: Extending the clinical phenotype.

BACKGROUND/OBJECTIVES: Congenital hemangiomas (CH) are a group of benign vascular tumors that are present at birth and exhibit variable involution during infancy. Congenital hemangiomas that do not involute are typically solitary patch or plaque-type tumors that grow proportionally with somatic growth. We report a case series of 9 patients with persistent CH, which exhibited uncommon features including segmental involvement, recurrent or severe pain, or growth via volumetric increase in size or apparent increased extent of anatomic involvement over time. METHODS: Via retrospective chart review, we included patients with persistent CH and atypical presentations. Available data regarding clinical characteristics, natural history, histopathology, imaging, and genetic tests were collected. RESULTS: Data on 9 patients were collected, including 7 noninvoluting CH and 2 partially involuting CH. Three of the 9 cases had segmental distribution, 6 had apparent growth or clinical evolution, and 4 were symptomatic with pain. One also had marked localized intravascular coagulopathy. CONCLUSIONS: Ongoing or recurrent pain and large extent of anatomic involvement can be features of CH, albeit uncommon ones, and can pose both diagnostic and management challenges. Tissue genomic studies can offer a novel tool for CH diagnosis.

Trichodysplasia spinulosa in a child: Identification of trichodysplasia spinulosa-associated polyomavirus in skin, serum, and urine.

A 6-year-old girl with a history of chronic immunosuppression following small bowel and colon transplantation for tufting enteropathy presented with a diffuse, facial-predominant eruption composed of pink-to-skin-colored papules with central white dystrophic spicules. Histology from a punch biopsy and polymerase chain reaction (PCR) from plucked spicules confirmed a diagnosis of trichodysplasia spinulosa (TS). Additional molecular studies identified several strains of the trichodysplasia spinulosa-associated polyomavirus infecting multiple tissues of the patient, confirming the systemic nature of trichodysplasia spinulosa infections.

Basan gets a new fingerprint: Mutations in the skin-specific isoform of SMARCAD1 cause ectodermal dysplasia syndromes with adermatoglyphia.

Basan syndrome is an autosomal dominant ectodermal dysplasia (ED) with congenital adermatoglyphia, transient neonatal acral bullae, and congenital facial milia. Autosomal dominant adermatoglyphia (ADG) is characterized as adermatoglyphia with hypohidrosis. Recently mutations in the skin-specific isoform of the gene SMARCAD1 have been found in both syndromes. This report proposes to unify these two previously distinct ED, into one syndrome. We offer a new acronym: SMARCAD syndrome (SMARCAD1-associated congenital facial Milia, Adermatoglyphia, Reduced sweating, Contractures, Acral Bullae, and Dystrophy of nails). Sanger sequencing was performed on genomic DNA from a patient with Basan syndrome using primers designed to flank SMARCAD1. Sanger sequencing revealed a novel variant, NM_001254949.1:c.-10 + 2 T > G, in the donor splice site of exon 1 of the skin-specific isoform. This variant and the other five previously reported variants in Basan syndrome and ADG are all within the same donor splice site. We conclude that Basan syndrome and ADG are on a phenotypic spectrum of a monogenic syndrome which is better described by the acronym SMARCAD syndrome.