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Purpose of Review: Provide an overview of recent findings and recommendations for the presentation, diagnosis, and management of pediatric dermatologic emergencies., Recent Findings: Updated information on optimal diagnosis and treatments for pediatric dermatologic emergencies because of infection, reactive infectious mucocutaneous eruption, and serious cutaneous adverse reactions are covered., Summary: Pediatric dermatologic emergencies require prompt diagnosis and management. This review covers new agents and techniques for management and diagnosis., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background and Objective: As the population and life expectancy of people with Down syndrome increases, identifying common skin conditions throughout the lifespan will help inform clinical care and research. We sought to evaluate dermatologic conditions diagnosed in pediatric and adult patients with Down syndrome., Methods: This multicenter retrospective study analyzed demographic and outpatient visit International Classification of Diseases codes of patients with Down syndrome evaluated at outpatient dermatology clinics in the United States or Canada between 2011 and 2021., Results: A total of 1529 patients with Down syndrome were identified from eight academic medical centers: 50.8% were children (0-12 years), 25.2% were adolescents (13-17 years), and 24% were adults (18 years and older). Eczematous dermatitis was the most common diagnosis overall (26%), followed by folliculitis (19.3%) and seborrheic dermatitis (15.6%). Other notable diagnoses included dermatophyte infections (13%), alopecia areata (11.6%), and psoriasis (6.7%). About 4.3% of visits included a code for high-risk medication use. Eczematous dermatitis, alopecia areata, and folliculitis were the most common diagnoses observed in children; folliculitis, hidradenitis suppurativa, and eczematous dermatitis in adolescents; and seborrheic dermatitis, eczematous dermatitis, and folliculitis in adults., Conclusions: Dermatologic conditions in patients with Down syndrome vary by age, but are most often eczematous, adnexal, and cutaneous autoimmune disorders. This multicenter retrospective review identifies skin diseases that should be prioritized for clinical care guideline development and research in the Down syndrome community., (© 2024 Wiley Periodicals LLC.)

Isotretinoin, the standard treatment for severe nodular acne, is subject to stringent iPLEDGE regulations due to its teratogenic risks, requiring monthly assessments for patients of childbearing potential. Analysis of the iPLEDGE Comprehension Assessment (iPCA) revealed an average readability score of grade 8.5, exceeding the recommended grade 6 level for optimal patient comprehension. The complex language of iPCA may hinder patients from accessing treatment, contributing to delays and potential discontinuation, especially among female patients. While the overall number of isotretinoin-exposed pregnancies has decreased since the inception of iPledge, several hundred pregnancies continue to be reported, and simplification of iPCA presents one avenue to improve patient comprehension, safety, and ensuring equitable access to isotretinoin., (© 2024 Wiley Periodicals LLC.)

The expertise of both dermatology and rheumatology may be beneficial when managing autoimmune conditions with cutaneous and systemic manifestations in children. This survey study was directed to pediatric dermatologists who participate in combined pediatric dermatology-rheumatology clinics; 13 sites in North America responded. The results provide information regarding clinic operations, benefits, and barriers to establishment. These findings have the potential to help institutions establish or modify combined pediatric dermatology-rheumatology clinics, although further research is needed to determine their impact., (© 2024 Wiley Periodicals LLC.)

Competing Interests: Conflicts of interest Dr Brandling-Bennett has served as principal investigator for Lilly. Dr Arkin has received consulting fees from AbbVie, Regeneron, and Verrica. Dr Hebert has received research grants (paid to UTHealth McGovern Medical School) from LEO Pharma, Mayne Pharma, UCB, GSK, Ortho Dermatolgics, Amgen, Arcutis, AbbVie, and Pfizer; has received honoraria from Pfizer, Arcutis, UCB, Verrica, LEO Pharma, Novan, Incyte, Janssen, Mayne Pharma, and Castle Creek Bioscience; and has served on drug safety monitoring boards for Ortho Dermatologics, GSK, and Sanofi Regeneron. Dr Cordoro has served as a member of a scientific steering committee for Celgene. Dr Curran has served as an advisory board member for Novartis, receiving honoraria. Dr Dalrymple has served as an advisory board member for Novartis. Dr Flohr is chief investigator of the UK National Institute for Health Research-funded TREAT (ISRCTN15837754, EudraCT 2,015-002,013-29) and SOFTER (Clinicaltrials.gov: NCT03270566) trials as well as the UK-Irish Atopic Eczema Systemic Therapy Register (A-STAR; ISRCTN11210918) and a principal investigator in the European Union (EU) Horizon 2020-funded BIOMAP Consortium (http://www.biomap-imi.eu/). He also leads the EU Trans-Foods Consortium. His department has received funding from Sanofi-Genzyme and Pfizer for host-microbiome research. Dr Gordon has received personal fees from AbbVie, Almirall, Amgen, BMS, Dermavant, Dermira, Lilly, Janssen, Kyowa Hakko Kirin, LEO Pharma, Novartis, Pfizer, Sun Pharma, and UCB and grants from BMS, Lilly, and UCB. Dr Irvine has received person fees from AbbVie, LEO Pharma, Lilly, Novartis, and Sanofi/Regeneron, and has received a grant from the UK National Institute for Health Research (research arm of the UK Department of Health) as co-principal investigator of a clinical trial comparing MTX with cyclosporine in children with severe atopic dermatitis. Dr Kirkorian has received honoraria from Verrica. Dr Lara-Corrales has served as an advisory board member for Ipsen, LEO Pharma, Lilly, Novartis, Pfizer, and Sanofi Genzyme; as a consultant for AbbVie, Avicanna, Janssen, Johnson & Johnson, Loreal, Pfizer, Pierre Fabre, Sanofi Genzyme, and Valeant; as a speaker for AbbVie, Amgen, Novartis, and Sanofi Genzyme; and has served as a site investigator for AbbVie, Clementia Pharmaceuticals, Janssen, Lilly, and, Mayne Pharma. Dr Jill Lindstrom has received consulting fees from AbbVie, AnaptysBio, Arena Pharmaceuticals, Aristea, Incyte, and Priovant. Dr Amy S. Paller has served as an investigator for AbbVie, Applied Pharma Research, Dermavant, Eli Lilly, Incyte, Janssen, Krystal, Regeneron, and UCB; Consultant for Aegerion Pharma, Azitra, BioCryst, Boehringer-Ingelheim, Bristol Myers Squibb, Castle Creek, Eli Lilly, Janssen, Krystal, LEO Pharma, Novartis, Regeneron, Sanofi/Genzyme, Seanergy, TWI Biotechnology, and UCB, and on the data safety monitoring board for AbbVie, Abeona, Catawba, Galderma, and InMed. Author Begolka has served as an advisory board member for Incyte and Pfizer, and has received a grant from Pfizer. Dr Tom has served as a site investigator for clinical trials for Janssen, Regeneron, Incyte, Pfizer, Dermira, Eli Lilly, and AbbVie, and has been on data safety committees for LEO Pharma. Dr Voorhees has received research grants from AbbVie, Celgene, and Lilly; has served as an advisory board member for BMS, Boehringer Ingelheim, Celgene, Novartis, and UCB; and had received consulting fees from Amgen. Dr Vleugels has received consulting fees from AbbVie, Pfizer, and Priovant. Dr Lee has received consulting fees from AbbVie and Krystal Biotech; served as an investigator for AbbVie, Amryt, Arcutis, BMS, Castle Creek Biosciences, Celgene, Galderma, Incyte, Janssen, Kiniksa, Lilly, Mayne Pharma, Novartis, Pfizer, Regeneron, Sanofi Genzyme, Target Pharma, Trevi Therapeutics, and UCB; and has served as an advisory board member for Lilly, Novartis, Pfizer, and Regeneron. Dr Davies has received a fellow stipend from Pediatric Dermatology Research Alliance (PeDRA). Dr Siegfried has received consulting fees from AbbVie, Boehringer Ingelheim, Incyte, LEO Pharma, Novan, Novartis, Pierre Fabre, Pfizer, Regeneron, Sanofi Genzyme, UCB, and Verrica; has received honoraria from Regeneron, Sanofi Genzyme, and Verrica; has served on data safety monitoring boards for LEO Pharma, Novan, Pfizer, and UCB; participated in contracted research for AI Therapeutics; served as principal investigator for Janssen, and her institution has received fees related to clinical trials from Janssen, Lilly, Pierre Fabre, Regeneron, and Verrica and in support of a 2020-2021 pediatric dermatology fellowship from Pfizer. Dr Chiu, Dr Callen, Dr Castello-Soccio, Dr Co, Author Hanna, Dr Kim, and Dr Reyes have no conflicts of interest to declare.

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., (© 2024 Wiley Periodicals LLC.)

Competing Interests: None disclosed.

Pediatric cutaneous T-cell lymphoma with γδ immunophenotype is extremely rare. Only a few cases of γδ T-cell neoplasm have been reported in the literature, and therefore little is known whether γδ T-cell neoplasms in children are distinct from their adult counterparts with respect to the clinicopathological presentation, behavior, and prognosis. In this study, we demonstrate three unique pediatric cutaneous T-cell neoplasm and mimics with increased γδ T cells. All cases showed an indolent clinical course., (© 2024 Wiley Periodicals LLC.)

Competing Interests: Conflicts of interest Dr Lara-Corrales has received honoraria from Pierre Fabre, Amgen, Ipsen, Novartis, Pfizer, and Sanofi Genzyme and grants from AbbVie, Janssen, Clementia, Eli Lilly, Mayne Pharma, and Sanofi Genzyme. Dr Liy-Wong is advisor for Sanofi, Bayer, and Pfizer. Dr McKenzie is advisor for AbbVie, Amgen, Bausch, Bristol-Myers, Celgene, Galderma, Janssen, Leo Pharma, Lilly, Novartis, Pfizer, Sandoz, Sanofi, Sun Pharma, and UCB. Dr Rieder is advisor/consultant for, has received grants/honoraria from, and/or has served as a speaker for LEO Pharma, Pfizer, and Sanofi Genzyme. Drs Martinez-Cabriales, Coulombe, Aaron, Hussain, Linggonegoro, Barootes, Brandling-Bennett, Covelli, Kirkorian, Shah, Castelo-Soccio, Arkin, Heinze, Travis, Del Pozzo-Magana, Schoch, Monir, Glick, Uwakwe, Skillman, Hekman, Lethebe, and Ramien have no conflicts of interest to declare.

Mucocutaneous eruptions are associated with numerous infectious processes and can present as erythema multiforme (EM), reactive infectious mucocutaneous eruption (RIME), Stevens Johnson syndrome (SJS), or toxic epidermal necrolysis (TEN). Limited reports have detailed the association of these eruptions with SARS-CoV-2 infection. We present a series of eight cases of severe mucocutaneous blistering eruptions associated with SARS-CoV-2 infection. A retrospective case series was performed at six tertiary medical centers from March 1, 2020 to August 1, 2022. Inclusion criteria were met with a clinical diagnosis of EM, RIME, SJS, or TEN and a positive SARS-CoV-2 test (rapid antigen or polymerase chain reaction) less than 4 weeks prior to onset of dermatologic manifestation. Data was collected at time of each patient encounter. Eight patients met criteria with six pediatric patients (<18 years of age) having a median age of 15 years and two adult patients (>18 years of age) having a median age of 36 years. Patients were found to have a clinical diagnosis of RIME in 85.7% of cases. Oral mucosal involvement was the most common clinical finding (100%), followed by ocular (50.0%), urogenital (50.0%), and skin (37.5%) involvement. Evaluation did not reveal any additional infectious triggers in four patients. Evidence of possible concurrent or previous infectious triggers were identified in four patients. This case series highlights the development of severe mucocutaneous eruptions in association with COVID-19 infection, as well as the potential contributing role of concurrent or prior infections., (© 2023 The Authors. Pediatric Dermatology published by Wiley Periodicals LLC.)

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., Competing Interests: Conflicts of interest Dr Faith is a consultant for Verrica Pharmaceuticals, Novartis, and Canfield Scientific. Other authors have no conflicts of interest to declare., (Copyright © 2023 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Methotrexate (MTX) is a readily accessible drug, first used in 1948 and employed for a wide variety of indications since then. However, despite widespread off-label use, FDA labeling does not include approved indications for the use of MTX for many inflammatory skin diseases in pediatric patients, including morphea, psoriasis, atopic dermatitis, and alopecia areata, among others. Without published treatment guidelines, some clinicians may be hesitant to use MTX off-label, or uncomfortable prescribing MTX in this population. To address this unmet need, an expert consensus committee was convened to develop evidence- and consensus-based guidelines for use of MTX to treat pediatric inflammatory skin disease. Clinicians with experience and expertise in clinical research, drug development, and treating inflammatory skin disease in pediatric patients with MTX were recruited. Five committees were created based on major topic areas: (1) indications and contraindications, (2) dosing, (3) interactions with immunizations and medications, (4) adverse effects (potential for and management of), and (5) monitoring needs. Pertinent questions were generated and addressed by the relevant committee. The entire group participated in a modified Delphi process to establish agreement on recommendations for each question. The committee developed 46 evidence- and consensus-based recommendations, each with >70% agreement among members, across all five topics. These are presented in tables and text, along with a discussion of supporting literature, and level of evidence. These evidence- and consensus-based recommendations will support safe and effective use of MTX for the underserved population of pediatric patients who may benefit from this valuable, time-honored medication., (© 2023 The Authors. Pediatric Dermatology published by Wiley Periodicals LLC.)

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., (© 2022 Wiley Periodicals LLC.)

Competing Interests: Conflicts of interest None disclosed.

Competing Interests: Conflicts of interest None disclosed.

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., (© 2022 Wiley Periodicals LLC.)

Congenital pigmentary anomalies may be evident at birth or soon after, with some birthmarks becoming apparent later in infancy or early childhood. It is important to recognize various pigmentary anomalies in the neonate, most of which are benign but a subset of which are associated with cutaneous morbidity or systemic ramifications and require further evaluation. This review will focus on pigmentary mosaicism, congenital melanocytic nevi, nevus spilus, dermal melanocytosis, and pigmentary anomalies associated with neurofibromatosis type 1 (café au lait spots, freckling, plexiform neurofibromas, nevus anemicus), tuberous sclerosis (hypomelanotic macules), and incontinentia pigmenti., (Copyright © 2021 by the American Academy of Pediatrics.)

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., (© 2021 Wiley Periodicals LLC.)

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., (© 2021 Wiley Periodicals LLC.)

Importance: Hidradenitis suppurativa (HS) in pediatric patients has been understudied. Increased awareness and recognition of HS prevalence in children demand efforts to better understand this condition., Objective: To describe the demographics, clinical features, treatment, associated comorbidities, and outcomes in a large cohort of pediatric patients with HS., Design, Setting, and Participants: International, multicenter, retrospective medical record review of pediatric patients (aged 1-18 years) with a clinical diagnosis of HS carried out in 10 dermatology clinics across the US, Canada, Israel, Australia, and Italy from January 1996 to January 2017., Main Outcomes and Measures: Patient demographics, clinical features, severity, associated comorbidities, and treatments in pediatric patients with HS., Results: This cross-sectional study included 481 patients diagnosed with HS. Overall, 386 (80%) were girls. The mean (SD) age of disease onset was 12.5 (2.9) years, and the mean (SD) age at diagnosis was 14.4 (3.5) years. Family history of HS was present in 111 of 271 (41%) patients. First signs/symptoms reported at disease onset were cyst/abscess in 229 of 481 (48%), pain/tenderness in 118 of 481 (25%), and papules/pustules in 117 of 481 (24%). At initial dermatologic assessment, 233 of 481 (48%) patients already had evidence of skin scarring. Disease severity (Hurley staging) was documented in 288 of 481 (60%) patients (47% stage 1, 45% stage 2 and 8% stage 3). Comorbid conditions were reported in 406 of 481 (85%) patients, the most common being obesity (263/406 [65%]) and acne vulgaris (118/406 [29%]). Complications occurred in 378 of 481 (79%) patients, the most common of which were scars or contractures (301/378 [80%])., Conclusions and Relevance: The findings of this study indicate that there is a gap in recognizing and diagnosing pediatric HS. Pediatric patients with HS are likely to present with other comorbidities. Prospective observational and interventional studies are needed to better understand clinical course and optimal treatments for pediatric HS.

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.

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.

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., (© 2019 Wiley Periodicals, Inc.)

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., (© 2019 Wiley Periodicals, Inc.)

Background: Management of inpatient skin disease represents a unique subspecialty within dermatology., Objective: To assess the national burden of inpatient dermatology in adults., Methods: Using the 2014 National Inpatient Sample, we performed a retrospective cohort study of adults hospitalized for dermatologic conditions., Results: In 2014, there were 644,320 weighted hospitalizations principally for skin disease in adults, which cost the health care system $5.04 billion. Overall, skin disease was diagnosed in 1 in 8 hospitalized adults. Dermatologic hospitalizations were associated with a lack of medical insurance (odds ratio [OR], 2.27; 95% confidence interval [CI], 2.20-2.34), residence in a low-income community (OR, 1.10; 95% CI, 1.07-1.13), and small (OR, 1.27; 95% CI, 1.23-1.32) or rural hospitals (OR, 1.38; 95% CI, 1.32-1.44). Racial minorities were less likely to be hospitalized for skin disease than were whites (for blacks: OR, 0.77; 95% CI, 0.75-0.79; for Hispanics: OR, 0.85; 95% CI, 0.83-0.8; for Asians: OR, 0.59; 95% CI, 0.55-0.64). Only 0.47% of patients admitted for skin disease experienced in-hospital mortality; however, mortality rates were high in hospitalizations for cutaneous lymphomas (9.19%) and malignant melanoma (6.54%)., Limitations: We could not assess the impact of inpatient dermatology consultations on hospitalization outcomes., Conclusions: Skin disease is highly prevalent among hospitalized patients., (Copyright © 2018 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Birthmarks are common in the healthy population and are generally harmless. Certain presentations, however, raise concern for associated syndromes or potential complications. It is important for pediatricians to be familiar with both harmless and potentially concerning birthmarks. This article discusses congenital melanocytic nevi, café-au-lait macules, hypomelanotic macules, nevus depigmentosus, nevus anemicus, epidermal nevi, and nevus sebaceous, including potential syndromes and complications. [Pediatr Ann. 2019;48(1):e23-e29.]., (Copyright 2019, SLACK Incorporated.)

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&#95;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., (© 2018 Wiley Periodicals, Inc.)

The extrathyroid manifestations of Graves disease (GD) include thyroid orbitopathy, dermopathy, and acropachy. Thyroid dermopathy (TD), also known as pretibial myxedema, classically presents as nonpitting edema or plaquelike lesions on the pretibial region, while thyroid acropachy (TA) is seen in cases of severe TD, characterized by soft tissue swelling and clubbing of fingers and toes, as well as a periosteal reaction of the bones of the hands and feet. Both TD and TA are rare manifestations of thyroid disease and uncommonly reported in pediatric patients. Our aim was to increase awareness of dermatological manifestations associated with pediatric GD and review the literature of pediatric thyroid dermopathy as well as report a case of acropachy in a child., (© 2018 Wiley Periodicals, Inc.)

Background: Hospital readmissions represent a potential target for reducing unnecessary health care expenditures; however, readmissions following dermatology hospitalizations remain poorly characterized., Objective: To assess the frequency and demographics of readmissions for skin disease., Methods: We performed a retrospective cohort study of dermatology hospitalizations by using the 2014 Nationwide Readmissions Database., Results: Readmissions following dermatologic hospitalizations cost the American health care system $1.05 billion in 2014. The 30-day rate of all-cause readmission following the 647,251 weighted index admissions for skin disease was 12.63%. Readmission was most common following hospitalizations for cutaneous lymphomas (39.63%), connective tissue disorders (26.28%), and cutaneous congenital abnormalities (23.86%). Predictors of readmission included public insurance with Medicaid (odds ratio [OR], 1.61; 95% confidence interval [CI], 1.53-1.70) or Medicare (OR, 1.55; 95% CI, 1.48-1.62), residence in a low-income community (OR, 1.14; 95% CI, 1.09-1.20), an increased number of chronic conditions (OR, 4.46; 95% CI, 4.15-4.79), and a large hospital (OR, 1.10; 95% CI, 1.05-1.16). Urban (OR, 0.90; 95% CI, 0.87-0.94) and rural (OR, 0.78; 95% CI, 0.73-0.82) nonteaching hospitals were protective against readmissions from skin disease., Limitations: We were unable to assess the impact of inpatient dermatology consultations on hospital readmission rates., Conclusions: There are significant health care and demographic disparities in readmissions for skin disease., (Copyright © 2018 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Background/objectives: It is known that inpatient care accounts for a significant portion of health care expenditures, but the national burden of inpatient pediatric dermatology is poorly characterized. We sought to assess risk factors, conditions, and financial costs associated with pediatric hospitalizations for skin disease., Methods: We performed a cross-sectional study of pediatric dermatology hospitalizations using the 2012 Kids' Inpatient Database, which samples 80% of non-birth-related pediatric admissions from 44 states to generate national estimates. The demographic characteristics of children admitted for dermatologic and nondermatologic conditions were compared, and the financial costs of these admissions were analyzed., Results: In 2012, there were 74 229 (95% confidence interval (CI) = 68 620-79 978) pediatric dermatology hospitalizations, accounting for 4.2% of all pediatric admissions and $379.8 million (95% CI = $341.3-418.4 million) in health care costs. Bacterial infections (n = 59 115, 95% CI = 54 669-63 561), viral diseases (n = 3812, 95% CI = 3457-4167), and noncancerous skin growths (n = 2931, 95% CI = 2318-3545) were the most common conditions requiring hospitalization. The highest mean cost per hospitalization was for admissions for cutaneous lymphomas ($58 294, 95% CI = $31 694-84 893), congenital skin abnormalities ($24 186, 95% CI = $16 645-31 728), and ulcers ($17 064, 95% CI = $14 683-19 446). Pediatric dermatology hospitalizations were most strongly associated with living in a low-income community (odds ratio (OR) = 1.22, 95% CI = 1.16-1.29) and the South (OR = 1.32, 95% CI = 1.19-1.46) and being uninsured (OR = 1.35, 95% CI = 1.26-1.45) or having Medicaid insurance (OR = 1.17, 95% CI = 1.13-1.22)., Conclusion: Skin disease is a common cause of hospitalizations in children, and there are disparities in these admissions that could reflect inadequate access to outpatient pediatric dermatologists., (© 2018 Wiley Periodicals, Inc.)

Fibrous hamartoma of infancy (FHI) is a benign mesenchymal tumor of young children. It has a broad clinical differential diagnosis and is often clinically confused for vascular and malignant soft tissue neoplasms. Recognition of the unique histologic features of FHI, a triphasic population of mature adipose tissue, mature fibrous tissue, and immature mesenchymal tissue, will ensure the correct diagnosis. In this report we present a case of this rare entity, including the associated clinical, radiologic, and histologic findings.

A 4-year-old girl with autism spectrum disorder and congenital heart disease presented to dermatology clinic for evaluation of skin growths present since infancy. Physical examination was significant for macrocephaly and agminated skin-colored to pink papulonodules in a segmental distribution on the right lower back and buttocks, biopsy of which showed storiform collagenomas (sclerotic fibromas). Genetic testing revealed a pathogenic missense mutation in the PTEN gene, and a diagnosis of PTEN hamartoma tumor syndrome was made. The segmental nature of her storiform collagenomas is unique, to our knowledge, and may be explained by a postzygotic second-hit PTEN mutation, contributing to the growing spectrum of clinical findings associated with PTEN hamartoma tumor syndrome., (© 2018 Wiley Periodicals, Inc.)

Erythema ab igne (EAI) is an asymptomatic dermatosis caused by prolonged exposure to localized heat. Affected areas have net-like hyperpigmentation that may resemble more serious conditions such as livedo racemosa or vasculitis. We report three cases of EAI in pediatric sickle cell disease (SCD) patients who were initially suspected of having a more severe, life-threatening disorder before Dermatology was consulted. Clinicians caring for pediatric SCD patients who regularly use heating pads/devices for pain relief should consider EAI in the differential diagnosis of large areas of net-like hyperpigmentation. This paper aims to increase recognition of EAI and patient education on safe practices while using heating pads.

Acral pigmented Spitz nevi are seldom reported in the literature. We report a new case on the palm of a 4-year-old girl that demonstrated correlation between features observed on dermoscopy and reflectance confocal microscopy (RCM). Histopathology revealed a benign intraepidermal Spitz nevus with transepidermal elimination of melanocytes that showed on RCM as focal atypical bright cells concerning for malignancy. This case is one of few reports in the literature combining dermoscopy, reflectance confocal microscopy, and histology for an acral Spitz nevi, which are rarely evaluated by RCM given the thickness of the stratum corneum in acral sites., (© 2018 Wiley Periodicals, Inc.)