Background/Objective In spring 2020, high numbers of children presented with acral pernio-like skin rashes, concurrent with the coronavirus disease 2019 (COVID-19) pandemic. Understanding their clinical characteristics/ infection status may provide prognostic information and facilitate decisions about management. Methods A pediatric-specific dermatology registry was created by the Pediatric Dermatology COVID-19 Response Task Force of the Society for Pediatric Dermatology (SPD) and Pediatric Dermatology Research Alliance (PeDRA) and was managed by Children's Hospital of Philadelphia using REDCap. Results Data from 378 children 0-18 years entered into the registry between April 13 and July 17, 2020 were analyzed. Data were drawn from a standardized questionnaire completed by clinicians which asked for demographics, description of acral lesions, symptoms before and after acral changes, COVID-19 positive contacts, treatment, duration of skin changes, laboratory testing including SARS-CoV-2 PCR and antibody testing, as well as histopathology. 229 (60.6%) were male with mean age of 13.0 years (+/- 3.6 years). Six (1.6%) tested positive for SARS-CoV-2. Pedal lesions (often with pruritus and/or pain) were present in 96%. 30% (114/378) had COVID-19 symptoms during the 30 days prior to presentation. Most (69%) had no other symptoms and an uneventful course with complete recovery. Conclusions and Relevance Children with acral pernio-like changes were healthy and all recovered with no short-term sequelae. We believe these acral changes are not just a temporal epiphenomenon of shelter in place during the spring months of the first wave of the COVID-19 pandemic and may be a late phase reaction that needs further study.

Hereditary cancer syndromes that affect the skin can be divided into the following groups: (1) disorders that predispose patients to mucocutaneous malignancies; (2) disorders that predispose patients to mucocutaneous and extracutaneous malignancies; and (3) disorders that have cutaneous stigmata and predispose patients to extracutaneous malignancies. Genodermatoses with malignant potential that are discussed in this chapter include Bazex–Dupré–Christol syndrome, Beckwith–Wiedemann syndrome, Birt–Hogg–Dubé syndrome, Brooke–Spiegler syndrome, epidermodysplasia verruciformis, hereditary leiomyomatosis, Gardner syndrome, Muir–Torre syndrome and constitutional mismatch repair deficiency syndromes, Peutz–Jeghers syndrome, and PTEN hamartoma-tumour syndrome.

Background: Stiff skin syndrome (SSS) is a noninflammatory, fibrosing condition of the skin, often affecting the limb girdles.Objective: We present 4 new patients with SSS with largely unilateral, segmental distribution. To date, reported cases of SSS have been grouped based on generally accepted clinical and histopathologic findings. The purpose of this study was to analyze differences in clinical and histopathologic findings between previously reported SSS cases.Methods: This is a retrospective review of 4 new cases and 48 previously published cases of SSS obtained from PubMed search.Results: Of 52 total cases, 18 (35%) were segmentally distributed and 34 (65%) were widespread. The average age of onset was 4.1 years versus 1.6 years for segmental versus widespread SSS, respectively. Limitation in joint mobility affected 44% of patients with segmental SSS and 97% of patients with widespread SSS. Histopathologic findings were common between the 2 groups.Limitations: This was a retrospective study of previously published cases limited by the completeness and accuracy of the reviewed cases.Conclusions: We propose a distinct clinical entity, segmental SSS, characterized by a segmental distribution, later age of onset, and less severe functional limitation. Both segmental SSS and widespread SSS share common diagnostic histopathologic features.

In the past 2 decades, there has been enormous progress in determining the molecular bases of genodermatoses . This progress has expanded the interface between dermatology and genetics. Integration of clinical and molecular data has simplified disease classification and highlighted relationships among conditions. However, the recent explosion in genetic knowledge has not yet been fully incorporated into clinical dermatology practice or dermatology resident education. This article highlights strategies to overcome barriers and correct practice and educational gaps, enhancing the ability of dermatologists to diagnose, counsel, evaluate, and treat patients and families affected by genodermatoses.

Background: Patients with atopic dermatitis (AD) are prone to skin infections, with microbes such as Staphylococcus aureus suspected of contributing to pathogenesis. Bleach baths might improve AD by reducing skin microbial burden.Objective: We sought to characterize the microbiota of lesional and nonlesional skin in young children with AD and control subjects and compare changes after treatment with a topical corticosteroid (TCS) alone or TCS 1 dilute bleach bath.Methods: In a randomized, placebo-controlled, single-blinded clinical trial in 21 children with AD and 14 healthy children, lesional and nonlesional AD skin was examined at baseline and after 4-week treatment with TCS alone or TCS plus bleach bath. Microbial DNA was extracted for quantitative polymerase chain reaction of predominant genera and 16S rRNA sequencing.Results: At baseline, densities of total bacteria and Staphylococcus, including Staphylococcus aureus, were significantly higher at the worst AD lesional site than nonlesional (P = .001) or control (P < .001) skin; bacterial communities on lesional and nonlesional AD skin significantly differed from each other (P = .04) and from control (P < .001). After TCS 1 bleach bath or TCS alone, bacterial compositions on lesional skin normalized (P < .0001), resembling nonlesional skin, with microbial diversity restored to control skin levels.Limitations: The 4-week time period and/or the twice-weekly baths may not have been sufficient for additional impact on the cutaneous microbiome. More detailed sequencing may allow better characterization of the distinguishing taxa with bleach bath treatment.Conclusions: Treatment with a TCS cream suffices to normalize the cutaneous microbiota on lesional AD; after treatment, bacterial communities on lesional skin resemble nonlesional skin but remain distinct from control.

Neurocutaneous syndromes are a heterogeneous group of congenital and hereditary disorders with manifestations in the skin and the nervous system, usually together with ocular features that represent diagnostic clues and potential sources of morbidity. Dermatologists and ophthalmologists often need to work together in identifying and managing patients with these conditions; herein, we focus on classic and under-recognized neurocutaneous syndromes. We begin with autosomal dominant genodermatoses characterized by hamartomas and tumors in the skin, eyes, and central nervous system: neurofibromatosis type 1, tuberous sclerosis complex, and PTEN hamartoma-tumor syndrome. This is followed by a discussion of two mosaic disorders, Sturge-Weber syndrome and neurocutaneous melanocytosis. In addition to providing an update on clinical presentations and evaluation of patients with these conditions, we review recent insights into their pathogenesis, drawing attention to relationships among the diseases on a molecular level and implications regarding treatment. We also highlight the major features of other neurocutaneous syndromes that have ocular findings plus pigmentary, vascular, hyperkeratotic, adnexal, connective tissue, photosensitive, and inflammatory manifestations in the skin.

A new or changing melanocytic nevus in a child or adolescent often leads to concern in parents and physicians. To avoid undue alarm and unnecessary procedures, dermatologists should be aware of the natural history and clinical spectrum of nevi in pediatric patients, as well as findings that are potentially worrisome in this age group. This review provides an update on melanocytic nevi in children, focusing on their dynamic evolution over time, molecular insights into nevogenesis, and phenotypic markers for increased risk of melanoma in adolescence and adulthood. Special considerations for Spitz nevi and nevi located in particular sites (eg, scalp, acral, genital) are highlighted. Current understanding of the risks associated with congenital melanocytic nevi of different sizes and strategies for the management of children with numerous acquired nevi, Spitz nevi, and congenital nevi are also discussed.

Facial involvement represents a characteristic feature of a wide range of genodermatoses. Specific facial findings often help point to the correct diagnosis, which improves counseling and management. In particular, this can facilitate the identification and treatment of associated extracutaneous disease. The highly visible nature of facial lesions in genodermatoses and facial birthmarks can result in stigmatization and frequently leads to particular concern in patients and their family members. It is therefore critical for dermatologists to be aware of the broad spectrum of facial manifestations in genetic skin disease, especially when these findings have important implications with regard to monitoring and treatment. In this contribution, facial involvement in genodermatoses is divided into five morphologic categories based on the most prominent feature: Papules, scaling, photosensitivity/findings associated with aging (eg, telangiectasias, atrophy, lentigines), blisters/erosions, and birthmarks. Hopefully, this will provide a practical and clinically useful approach to a large and diverse assortment of genetic skin conditions.

Genital melanocytic nevi in children are little studied and are usually excluded from large studies of pediatric nevi. Investigators retrospectively

Background: Little is known about the prevalence and clinical characteristics of genital melanocytic nevi in children.Objective: We sought to describe the epidemiology, clinical and dermoscopic features, and natural history of genital nevi in pediatric patients.Methods: We reviewed charts of 1159 children given the diagnosis of melanocytic nevi over 11 years. Those with genital nevus as a chief symptom were contacted for follow-up.Results: Among children/adolescents evaluated for nevi, the prevalence of genital nevus was 3.5% (40/1159), with a male: female ratio of 1.3: 1. There were no statistically significant differences in age, sex, total nevus number, presence of acral and scalp nevi, or family history of dysplastic nevi and melanoma between patients with and without genital nevi. Genital nevus onset was before age 2 years in 63.6% of patients. A globular dermoscopic pattern was observed in 93.3%. Most genital nevi underwent a gradual change in diameter, elevation (becoming soft papules), color, texture, or a combination of these. After median follow-up of 1.5 years, no melanoma or other adverse outcome was observed. Limitations: This was a retrospective chart analysis and questionnaire-based study of a limited number of patients.Conclusions: Increased awareness of the clinical characteristics, dermoscopic features, and evolution of genital nevi in children may help to avoid unnecessary surgery.

BACKGROUND:Congenital erosive and vesicular dermatosis (CEVD) healing with reticulated supple scarring, a condition usually observed in premature neonates, presents at birth with vesicles and erosions. Lesions typically heal within a few months, leaving behind scarring with a distinctive supple and reticulated texture.OBJECTIVES:We sought to merge existing literature with new cases to further define CEVD.METHODS:We analyzed 19 previous reports of CEVD and added 9 additional patients; we identified unifying characteristics of this cohort.RESULTS:In 28 total cases, notable features included: preterm birth (79%), nail abnormalities (46%), hyperthermia/hypohidrosis (46%), a history of maternal chorioamnionitis (43%), alopecia (43%), neurodevelopmental and ophthalmologic abnormalities (36% each), tongue atrophy (29%), or a combination of these. Patients with CEVD may be prone to postnatal herpetic superinfections. Previously unreported findings included: erosive lichen planus, digital tip gangrene, and hydronephrosis.LIMITATIONS:The small patient sampling makes it difficult to define diagnostic criteria. As certain findings are associated with prematurity, it is unclear to what extent these features result from CEVD, premature birth, or another intrauterine pathology.CONCLUSIONS:Although rare, CEVD should be considered in the differential diagnosis of neonatal vesicles/erosions in the context of a negative infectious workup. This review strengthens the spectrum of CEVD features, thus facilitating its recognition by clinicians.

IMPORTANCEControversy exists regarding strategies for diagnosis and management of Spitz nevi, a type of melanocytic neoplasm that most often develops in children.OBJECTIVETo determine the beliefs, behaviors, and experiences of pediatric dermatologists with regard to Spitz nevi.DESIGNAnonymous web-based survey.SETTINGPrivate and academic dermatology practices.PARTICIPANTSRespondents included 175 pediatric dermatologists from the United States and around the world, representing a 51.1% response rate (175 of 342). Analyses were limited to the 144 respondents whose practices included at least 50% children (younger than 18 years).MAIN OUTCOME MEASURESAssessment of the following with regard to Spitz nevi: frequency of diagnosis, general beliefs, techniques used for evaluation (eg, dermoscopy and biopsy), management strategies, and observed outcomes.RESULTSCollectively, respondents had seen approximately 20 000 Spitz nevi; 67.6% (96 of 142) had diagnosed at least 6 Spitz nevi yearly, whereas 90.1% (128 of 142) had diagnosed no more than 2 prepubertal melanomas in the past 5 years. Ninety-six percent of respondents (95.8%; 136 of 142) categorized typical Spitz nevi as benign. Eighty percent of respondents (79.6%; 113 of 142) used dermatoscopy, and 96.5% (137 of 142) avoided partial biopsies of Spitz nevi. In children with a suspected Spitz nevus, clinical follow-up was chosen by 49.3% (69 of 140) of respondents for a small, stable nonpigmented lesion and by 29.7% (41 of 138) for a pigmented lesion with a typical starburst pattern seen via dermatoscopy. Predictors of clinical follow-up of the latter lesion included believing that Spitz nevi are not melanoma precursors (P = .04). Forty-seven percent (62 of 132) of respondents had observed involution of Spitz nevi. No deaths had resulted from the approximately 10 000 Spitz nevi or atypical spitzoid neoplasms seen by the 91 respondents with academic or hospital-based practices.CONCLUSIONS AND RELEVANCEThe results of our survey support conservative management of Spitz nevi in children, with clinical follow-up representing an option for typical lesions. This represents an important difference from strategies used for management of these lesions in adults.

OBJECTIVE:To characterize the atypical cutaneous presentations in the coxsackievirus A6 (CVA6)-associated North American enterovirus outbreak of 2011-2012. METHODS:We performed a retrospective case series of pediatric patients who presented with atypical cases of hand, foot, and mouth disease (HFMD) from July 2011 to June 2012 at 7 academic pediatric dermatology centers. Patients were included if they tested positive for CVA6 or if they met clinical criteria for atypical HFMD (an enanthem or exanthem characteristic of HFMD with unusual morphology or extent of cutaneous findings). We collected demographic, epidemiologic, and clinical data including history of skin conditions, morphology and extent of exanthem, systemic symptoms, and diagnostic test results. RESULTS:Eighty patients were included in this study (median age 1.5 years, range 4 months-16 years). Seventeen patients were CVA6-positive, and 63 met clinical inclusion criteria. Ninety-nine percent of patients exhibited a vesiculobullous and erosive eruption; 61% of patients had rash involving >10% body surface area. The exanthem had a perioral, extremity, and truncal distribution in addition to involving classic HFMD areas such as palms, soles, and buttocks. In 55% of patients, the eruption was accentuated in areas of eczematous dermatitis, termed "eczema coxsackium." Other morphologies included Gianotti-Crosti-like (37%), petechial/purpuric (17%) eruptions, and delayed onychomadesis and palm and sole desquamation. There were no patients with serious systemic complications. CONCLUSIONS:The CVA6-associated enterovirus outbreak was responsible for an exanthem potentially more widespread, severe, and varied than classic HFMD that could be confused with bullous impetigo, eczema herpeticum, vasculitis, and primary immunobullous disease.

To the Editor: In 2003, Annessi and colleagues 1 Annessi G. Paradisi M. Angelo C. Perez M. Puddu P. Girolomoni G. Annular lichenoid dermatitis of youth. J Am Acad Dermatol. 2003; 49: 1029-1036 Abstract Full Text Full Text PDF PubMed Scopus (47) Google Scholar described annular lichenoid dermatitis of youth (ALDY) as a new clinicopathologic entity in 23 Italian children and adolescents. The patients presented with annular patches and thin plaques with red-brown borders and central hypopigmentation that favored the flanks and groin. Characteristic histologic features included a band-like, polyclonal lymphocytic infiltrate in the papillary dermis and keratinocyte necrosis localized to the tips of elongated quadrangular rete ridges, which alternated with thinner rete ridges.

To the Editor: A 14-year-old girl presented with a 2-week history of skin lesions and arthritis affecting her elbows and knees. She had a 1-year history of perinuclear antineutrophil cytoplasmic antibody–positive (antimyeloperoxidase) pauci-immune focal segmental glomerulonephritis and recalled having similar cutaneous findings at the onset of her renal disease. Her medications included prednisone (15 mg every other day) and enalapril.

Study Design •Multi-institutional, retrospective, case series of a convenience sample of patients evaluated by pediatric dermatologists with atypical presentations of HFMD. Inclusion Criteria •Viral confirmation of CVA6 or met the clinical case criteria (see below). Exclusion Criteria •Presentation could be explained by another named illness •Presentation consistent with classic HFMD with <5% body surface area (BSA) involved. Clinical Case Criteria •Definite case: CVA6 confirmed via rtPCR in oropharynx, skin, blood, or stool •Probable case: At least 1 criterion from group 1, and at least 1 from group 2