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Background: High-risk congenital melanocytic nevi (CMN) are associated with abnormalities of the central nervous system (CNS), prompting magnetic resonance imaging (MRI) screening guidelines.Objective: Describe MRI brain and spine abnormalities in children with CMN and report trends between nevus features, MRI findings, and neurologic outcomes.Methods: Retrospective review of individuals aged ≤18 years with an MRI of the brain and/or spine and at least 1 dermatologist-diagnosed CMN.Results: Three hundred fifty-two patients were identified. Forty-six children had CMN that prompted an MRI of the brain and/or spine (50% male, average age at first image, 354.8 days). In these children, 8 (17%) had melanin detected in the CNS, of whom all had >4 CMN. One developed brain melanoma (fatal). In patients without CNS melanin, 4 had concerning imaging. Concerning MRI patients had more neurodevelopmental problems, seizures, neurosurgery, and death than individuals with unremarkable imaging. Three hundred six patients received MRIs for other reasons; none detected melanin. No children with only multiple small CMN (n = 15) had concerning imaging.Limitations: Lack of a control group, cohort size, and retrospective methods.Conclusion: MRI of the brain and spine is useful for detecting intervenable abnormalities in high-risk children. Healthy infants with few small CMN may not require screening MRI. [ABSTRACT FROM AUTHOR]

Background & Objective: Predicting the transformation of dysplastic or congenital nevi into malignant lesions results in a significant increase in the survival of patients. Some specific gene mutations have been reported to be very helpful in this regard. Therefore, this study aimed to evaluate the prevalence of BRAF V600E mutation in dysplastic and congenital nevi.Methods: This cross-sectional study was conducted on patients with congenital (n=30) or dysplastic (n=30) nevi. For genomic analysis, the BRAF gene mutation (V600E) was evaluated using the real-time polymerase chain reaction.Results: The prevalence of BRAF gene (V600E) mutation was found as 1 case (3.3%) in congenital and 8 cases (26.7%) in dysplastic nevi indicating the higher prevalence of this mutation in patients with dysplastic nevi (p =0.026). Moreover, in the dysplastic nevi group, the presence of BRAF gene mutation (V600E) showed a significant relationship with the severity of dysplasia as the mutation rate was 25% in mild cases, in comparison with 54.5% in moderate dysplasia cases (p =0.009).Conclusion: According to the results, 3.3% of the patients with congenital nevi and 26.7% of the subjects with dysplastic nevi were positive for BRAF V600E mutation. Furthermore, the severity of dysplasia could have a positive relationship with the presence of the mutation.

Abstract Giant melanocytic nevus is a rare condition caused by benign proliferation of melanocytes. There is a slight risk of malignancy in these lesions which should be noticed especially when they become larger. GCMN can be removed by plastic surgery.

Giant melanocytic nevus is a rare condition caused by benign proliferation of melanocytes. There is a slight risk of malignancy in these lesions which should be noticed especially when they become larger. GCMN can be removed by plastic surgery. [ABSTRACT FROM AUTHOR]

The port-wine stain in adults, or acquired port-wine stain, is a rare entity. A fifty-year-old female presented with complaints of an asymptomatic erythematous patch affecting the right side of the face for the previous twenty years. The lesion gradually increased in size to involve the area below the eye, right nasolabial fold, cheek adjacent to the nasolabial fold, and area between the right nasolabial fold and the upper lip. On the basis of clinical and dermoscopic examinations, the diagnosis of a port-wine stain was established. [ABSTRACT FROM AUTHOR]

Grouped patterns of pigmented lesions are infrequent. Of the several reports of agminated nevi, most have been Spitz nevi or blue nevi. The distribution of these nevi is often segmental, following a dermatome or the lines of Blaschko. Most segmental nevi are not agminated and develop early in childhood [1]. We describe a rare case of congenital agminated segmental nevi on the chest.

Background & Objective: Predicting the transformation of dysplastic or congenital nevi into malignant lesions results in a significant increase in the survival of patients. Some specific gene mutations have been reported to be very helpful in this regard. Therefore, this study aimed to evaluate the prevalence of BRAF V600E mutation in dysplastic and congenital nevi. Methods: This cross-sectional study was conducted on patients with congenital (n=30) or dysplastic (n=30) nevi. For genomic analysis, the BRAF gene mutation (V600E) was evaluated using the real-time polymerase chain reaction. Results: The prevalence of BRAF gene (V600E) mutation was found as 1 case (3.3%) in congenital and 8 cases (26.7%) in dysplastic nevi indicating the higher prevalence of this mutation in patients with dysplastic nevi (P=0.026). Moreover, in the dysplastic nevi group, the presence of BRAF gene mutation (V600E) showed a significant relationship with the severity of dysplasia as the mutation rate was 25% in mild cases, in comparison with 54.5% in moderate dysplasia cases (P=0.009). Conclusion: According to the results, 3.3% of the patients with congenital nevi and 26.7% of the subjects with dysplastic nevi were positive for BRAF V600E mutation. Furthermore, the severity of dysplasia could have a positive relationship with the presence of the mutation. [ABSTRACT FROM AUTHOR]

In inflammatory dermatoses, dermal melanophages (MLP) are ascribed to "pigment incontinence," with melanin "dropping down" from the epidermis. Although this is analogous to the "dropping down" of melanocytic nevus cells (Abtropfung), MLP in ordinary nevi have not been systematically studied—so "pigment incontinence" may not apply to MLP in nevi. A total of 31 childhood nevi identified by pediatricians and family practitioners were evaluated for the distribution of MLP. We tested the hypothesis that a dermal origin of the melanin in MLP is more likely than dropping down from the epidermis. In our cohort, 90.3% (28/31) of childhood nevi had dermal MLP, a significantly higher frequency, compared to 31/60 ordinary adult nevi (P < 0.0001). Superficial dermis was the most common location (P < 0.001). However, only six specimens had MLP restricted to the superficial dermis, significantly less than predicted by the theory that melanin drops down from the epidermis (P < 0.00001). We also evaluated perivascular MLP, since nerves run together with vessels in neurovascular bundles (NVB), and it has been showed that precursors of melanocytes migrate from the neural crest to the skin as nerve sheath stem cells. Superficial NVB MLP correlated with deep NVB bundle MLP (P < 0.05), suggesting that NVB MLP represent "tombstones" for superficial and deep dermal nevus cells. Deep dermal, deep NVB, and deep periadnexal MLP may be valid biological criteria for diagnosis of congenital type (prenatal) nevi. Viewing prenatal nevi in children as a neurocristopathy fits a major principle of pediatric pathology: childhood diseases should be studied and understood based on what happens during tissue development. [ABSTRACT FROM AUTHOR]

Competing Interests: Dr Correa-Selm is a consultant for Accutec and a consultant and researcher for Novartis Pharmaceutical, also serves on the Advisory Board for the Jacinto Convit World Organization and the Dermatology Advisory for Melanoma Research Foundation. Dr Grichnik is a consultant for Galileo Group and Canfield Scientific; serves on the Skin Advisory Board for Regeneron and the Dermatology Advisory for Melanoma Research Foundation; and receives clinical trial support from Novartis, Eli Lilly, Dermira, Elorac, Boehringer, and Amgen. Author Milani, Author Hanlon, and Dr Chen have no conflicts of interest to declare.

A 12-year-old girl presented to our attention for a pigmented lesion having greatest diameter of 2.5 cm, located on her left forehead, involving the ipsilateral eyebrow. This lesion had appeared as a flat brown macule at birth. With passing years, the lesion showed an increased diameter and thickness and it became progressively darker. 1. What reasons can justify the excision of congenital melanocytic nevi? 2. Which treatment do you think would be more appropriate? 3. Is there a right age to remove a congenital melanocytic nevus?

Congenital melanocytic nevi (CMN) are melanocytic lesions presenting at birth or appearing during the first weeks of life. CMN are usually grouped, according to size, in three categories: 1) small congenital nevi (SCN), measuring less than 1.5 cm in greatest diameter; 2) intermediate congenital nevi (ICN) that are 1.5-19.9 cm in greatest diameter; 3) large or giant nevi, larger than 20 cm in greatest diameter. Although the risk of a nevus evolving into a melanoma is low, all congenital nevi, particularly giant nevi, can be considered potential melanoma precursors. Furthermore, other criteria should be considered for an appropriate management of these lesions.

Congenital nevi develop before birth and sometimes cover large areas of the body. They are presumed to arise from the acquisition of a gene mutation in an embryonic melanocyte that becomes trapped in the dermis during development. Mice bearing the Cdk4 R24C ::Tyr- NRAS Q 61K transgenes develop congenital nevus-like lesions by post-natal day 10, from melanocytes escaping the confines of hair follicles. We interbred these mice with the collaborative cross ( CC), a resource that enables identification of modifier genes for complex diseases (those where multiple genes are involved). We examined variation in nevus cell density in 66 CC strains and mapped a large-effect quantitative trait locus ( QTL) controlling nevus cell density to murine chromosome 9. The best candidate for a gene that exacerbates congenital nevus development in the context of an NRAS mutation is Cdon, a positive regulator of sonic hedgehog (Shh) that is expressed mainly in keratinocytes. [ABSTRACT FROM AUTHOR]

Congenital nevi develop before birth and sometimes cover large areas of the body. They are presumed to arise from the acquisition of a gene mutation in an embryonic melanocyte that becomes trapped in the dermis during development. Mice bearing the Cdk4 R24C ::Tyr- NRAS Q 61K transgenes develop congenital nevus-like lesions by post-natal day 10, from melanocytes escaping the confines of hair follicles. We interbred these mice with the collaborative cross ( CC), a resource that enables identification of modifier genes for complex diseases (those where multiple genes are involved). We examined variation in nevus cell density in 66 CC strains and mapped a large-effect quantitative trait locus ( QTL) controlling nevus cell density to murine chromosome 9. The best candidate for a gene that exacerbates congenital nevus development in the context of an NRAS mutation is Cdon, a positive regulator of sonic hedgehog (Shh) that is expressed mainly in keratinocytes. [ABSTRACT FROM AUTHOR]

Résumé Introduction L’incidence des nævi congénitaux est de 1/20 000 nouveau-nés par an, 14 % des nævi congénitaux intéressent la région tête et cou. Les nævi de la région orbitaire sont particulièrement difficiles à prendre en charge sur le plan esthétique et fonctionnel. Les objectifs de cette étude étaient de réaliser une analyse des différentes présentations cliniques des nævi congénitaux de la région orbito-palpébrale chez l’enfant afin d’établir un algorithme thérapeutique. Patients et méthodes Nous avons réalisé une étude bi-centrique rétrospective incluant 51 enfants porteurs d’un nævus congénital de la région orbito-palpébrale. Nous avons étudié les formes cliniques, les traitements proposés et leurs résultats. Résultats Parmi les 51 patients, 19 ont bénéficié d’une exérèse plastie directe seule, trois d’une exérèse greffe de peau totale seule, 15 d’une combinaison de traitements dont quatre ont eu un expandeur et 14 étaient en abstention thérapeutique/surveillance. La durée moyenne de suivi était de 6,6 ans. Dans 33 cas, persistait une zone nævique résiduelle. Les séquelles postopératoires étaient : les dyschromies cicatricielles ( n = 17), les déformations anatomiques de l’œil ( n = 10), les résurgences næviques ( n = 8), les déformations canthales internes ( n = 5) et les ectropions ( n = 1). Conclusion L’évaluation de nos pratiques montre une place plus importante à l’abstention thérapeutique lorsque le préjudice esthétique est accepté par le patient et que le nævus ne présente pas de sur-risque de dégénérescence maligne. Afin de minimiser le risque de séquelles postopératoires nous proposons un algorithme thérapeutique dans la prise en charge des nævi congénitaux orbitaires. Summary Introduction The incidence of congenital nevi is one over 20,000 newborns per year, 14 % of them are located in the head and neck area. Nevi of the orbital region are particularly difficult to handle on the aesthetic and functional side. The objectives of this study were to conduct an analysis of different clinical presentations of congenital nevi of the eyelid orbital region in children to establish a treatment algorithm. Materiel and methods We realised a bi-centric retrospective study including 51 children with orbito-palpebral congenital nevi. We analysed the different clinical presentations, their treatments and their results. Results Nineteen underwent direct suture excision; three a total skin graft; 15 a combination of treatments, among them four underwent tissular expansion and 14 patients were not operated and clinically followed-up. The average follow-up time was 6.6 years. In 33 cases residual nevic area was still present. The postoperative sequelae were: dyschromia ( n = 17), anatomical deformation of the eye ( n = 10), nevi outbreaks ( n = 8), internal canthus deformation ( n = 5) and ectropion ( n = 1). Conclusion The results of our study show that therapeutic abstention is preferred when the aesthetic wrong is accepted by the patient and when there is not a higher risk of malignant degeneration. In order to minimise the risk of postoperative sequelae, we propose a therapeutic algorithm for the management of congenital orbital nevi. [ABSTRACT FROM AUTHOR]

Background Tissue expansion is a common reconstructive technique that has been associated with significant complications since its inception. However, the existing literature mostly focuses on complications associated with pediatric tissue expansion only or describes a combined population of adult and pediatric patients, including breast tissue expansion; despite the fact that each of these groups of patients has different characteristics that may affect tissue expansion. Objective In this study we present a critical review of our experience with complications of nonbreast tissue expansion in adult and pediatric patients and compare between these groups. Methods The charts of patients who underwent nonbreast tissue expansion at Hadassah Medical Center between January 2003 and July 2012 were reviewed. Data were collected including the age of the patient, anatomical site of the expansion, indication and complications. Results A total of 202 expansion procedures were performed on 119 pediatric patients (< 16 years) and 56 expansion procedures on 44 adult patients. The overall complication rate was 18.2%, with 40 pediatric procedures having complications (19.8%) and 7 adult procedures (12.5%). The difference in complication rates between the two groups was not found to be statistically significant. There was no statistically significant difference in complication rate between the different anatomical areas of expansion in both adult and pediatric patients or between the indications for operation. Most (68%) of the cases with complications underwent subsequent successful reconstruction. Conclusions Despite the consistent high complication rate, tissue expansion can be used as a good reconstructive method in both adult and pediatric patients in all anatomic areas and for different indications. [ABSTRACT FROM AUTHOR]

Neuro cutaneous melanocytosis (NCM) is a non-familial, congenital disorder characterized by multiple congenital nevi and brain or leptomeningeal abnormal melanin deposits. Here, we present an adult onset NCM. A 17-year-old boy presented with headache and double vision for 1 month. Magnetic resonance imaging of the brain showed hydrocephalus and abnormal meningeal hyper intensities in supra and infratentorial regions predominantly in the posterior fossa. Para medullary region showed an 11×10 mm nodular contrast enhancing nodule. Resection of an intramedullary central nervous system lesion revealed melanoma while skin biopsy was benign melanocytic nevus. As per Kadonaga and Frieden criteria, a diagnosis of NCM was made. Planned for craniospinal irradiation by three-dimensional conformal radiotherapy with a dose of 36 Gy, in 18 fractions (2 Gy/fraction and 5 days in a week) along with steroids however patient progressed and developed quadriplegia with intradural metastasis. [ABSTRACT FROM AUTHOR]

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Benign pigmented lesions are often treated for cosmetic reasons. Several modalities exist for the treatment of such lesions, with the aim being clearance of the lesion with acceptable cosmetic results. Cryotherapy and surgical excision of benign pigmented lesions result in inevitable scarring and the cosmetic end points may not be achieved. Such therapies may be declined by patients. The availability of lasers has made it possible to achieve clearance of these lesions with excellent cosmetic results. Although interest in such lasers has arisen in the past few decades, few studies comparing the efficacy of lasers and traditional treatment modalities exist. [ABSTRACT FROM AUTHOR]

Performing dermatologic surgery in infants and children presents unique challenges. A thorough understanding of both pediatric developmental milestones and their unique surgical circumstances is essential to providing optimal dermatologic care of the pediatric patient. In this work, we combine the authors’ experiences with data from the literature to provide pediatric dermatologic procedural pearls. [Copyright &y& Elsevier]

Abstract: Background: Neurocutaneous melanocytosis is a rare congenital dysplasia of the neuroectodermal melanocyte precursor cells that leads to proliferation of melanin-producing cells in the skin and leptomeninges. Patient: We describe a newborn with a giant congenital melanocytic nevus on his back, buttocks, and thighs. His brain magnetic resonance imaging study revealed bilateral T1 hyperintense lesions in the cerebellum and in the amygdala, hydrocephalus, and a Blake's pouch cyst, consistent with neurocutaneous melanocytosis and Dandy-Walker malformation. Conclusion: Neurocutaneous melanocytosis has a wide clinical spectrum that includes hydrocephalus, epilepsy, cranial nerve palsy, increased intracranial pressure, and sensorimotor deficits. [Copyright &y& Elsevier]

We present a 6-month-old male patient, who was consulted with dermatologist by his parents, because of a pigmented lesion, present since birth, covering almost the all skin of the back and buttocks. A sharply bordered, unequally coloured congenital pigmented nevus, measuring approximately 21 cm in diameter was observed in the whole body skin examination. The lesion was affecting the lower 2/3 of the skin of the back and the top half of the gluteus area, extending to the lateral part of the tors, forward the abdomen and the upper lateral part of the hips, composed by multiple darker-pigmented nests and several lighter areas, with single depigmented zones, hairy surface, irregularly infiltrated on palpation. Congenital melanocytic nevi are presented in approximately 1% of newborns, while giant congenital melanocytic nevi (GCMN) are the most uncommon subtype of them; with occurrence rate 1 in 50,000 births. They affect 2% of a total body surface or presenting in a diameter larger than 20 cm in older children. Although not common, the possible malignant transformation remains one of the most important considerations related to them, as the related lifetime risk of melanoma is 4% to 10%. Treatment recommendations include non-surgical methods as dermabrasion only within the first two weeks of life, for prevention the possible melanocytic deeper migration, while serial surgical excisions or tissue expanders could be useful treatment tool even in later stages. Nevertheless, cosmetic result is not always satisfactory, and the risk of malignant changes remains, in cases of previous melanocytic migration in deeper layer. Recent article suggests the potential role in the treatment of GCMN with NRAS inhibitor trametinib, approved for treatment of advanced melanoma, associated with underlying NRAS mutations. Although promising, the drug could be useful in paediatric patients, only with associated NRAS gene mutation. It is still unclear whether it could be helpful, independent of the NRAS status.

Congenital nevi develop before birth and sometimes cover large areas of the body. They are presumed to arise from the acquisition of a gene mutation in an embryonic melanocyte that becomes trapped in the dermis during development. Mice bearing the Cdk4(R24C) ::Tyr-NRAS(Q) (61K) transgenes develop congenital nevus-like lesions by post-natal day 10, from melanocytes escaping the confines of hair follicles. We interbred these mice with the collaborative cross (CC), a resource that enables identification of modifier genes for complex diseases (those where multiple genes are involved). We examined variation in nevus cell density in 66 CC strains and mapped a large-effect quantitative trait locus (QTL) controlling nevus cell density to murine chromosome 9. The best candidate for a gene that exacerbates congenital nevus development in the context of an NRAS mutation is Cdon, a positive regulator of sonic hedgehog (Shh) that is expressed mainly in keratinocytes., (© 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Introduction: The incidence of congenital nevi is one over 20,000 newborns per year, 14 % of them are located in the head and neck area. Nevi of the orbital region are particularly difficult to handle on the aesthetic and functional side. The objectives of this study were to conduct an analysis of different clinical presentations of congenital nevi of the eyelid orbital region in children to establish a treatment algorithm., Materiel and Methods: We realised a bi-centric retrospective study including 51 children with orbito-palpebral congenital nevi. We analysed the different clinical presentations, their treatments and their results., Results: Nineteen underwent direct suture excision; three a total skin graft; 15 a combination of treatments, among them four underwent tissular expansion and 14 patients were not operated and clinically followed-up. The average follow-up time was 6.6 years. In 33 cases residual nevic area was still present. The postoperative sequelae were: dyschromia (n=17), anatomical deformation of the eye (n=10), nevi outbreaks (n=8), internal canthus deformation (n=5) and ectropion (n=1)., Conclusion: The results of our study show that therapeutic abstention is preferred when the aesthetic wrong is accepted by the patient and when there is not a higher risk of malignant degeneration. In order to minimise the risk of postoperative sequelae, we propose a therapeutic algorithm for the management of congenital orbital nevi., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Melanoma in childhood is rare, and appears more commonly either in association with a preexisting (congenital) nevus, or with spitzoid features than de novo. Thus, problematic melanocytic lesions in children are essentially represented by congenital nevi and Spitz nevi that can be regarded as melanoma precursors and melanoma simulators, respectively. As a consequence, clinical and dermoscopic features of melanoma in children differ from those in an adult population. Herein we describe common clinical and dermoscopic features of problematic lesions in children, focusing on congenital and Spitz/Reed nevi, and including other problematic lesions, such as atypical, blue, acral, and scalp nevi., (Copyright © 2013 Elsevier Inc. All rights reserved.)