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Cutis verticis gyrata (CVG) is classified as primary or secondary according to the absence or presence of underlying soft tissue abnormalities. We report an infant with Turner syndrome (TS) who in addition presented with CVG on the scalp. The skin biopsy revealed a hamartoma-like lesion. We reviewed the clinical and histopathological findings of the 13 reported cases of congenital CVG in patients with TS, including ours. In 11 of them, CVG was localized on the skin of the scalp, mainly on the parietal region, and in two, on the forehead. Clinically, CVG had a flesh-colored aspect, with absent or sparse hair, and was not progressive. CVG was classified as primary in four patients who had skin biopsy and it was attributed to the intrauterine lymphedema of TS. However, histopathology in two of these patients identified dermal hamartoma as a secondary cause of CVG, and in three others, including ours, there were hamartomatous changes. Although further studies are required, previous findings support the proposal that some CVG may instead be dermal hamartomas. This report alerts clinicians to recognize CVG as a low-frequency manifestation of TS, but also to consider the possible co-occurrence of TS in all female infants with CVG.

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BACKGROUND: Cutaneous melanoma is the skin cancer with the highest mutational burden and metastatic rate. Early genetic alterations and biomarkers of distant progression are a point of interest. In addition to germline-susceptibility loci, almost 30% of melanomas arise from precursor benign nevi lesions, providing a source for malignant transformation. CASE PRESENTATION: Patient#009 developed a cutaneous melanoma over a nevus, followed by progression to regional and distant metastases in months, unresponsive to targeted therapy. To search for the genetic contribution to this rapid progression, a longitudinal analysis was performed through WES of germline, nevi, primary tumor, and a metastatic lymph node. Differential SNP/INDEL and CNV gene alterations, with functional impact on key pathways and cancer hallmarks in each step of evolution, were discerned. Tumor-associated nevus was, for the first time, split into two sections, distant and adjacent to the primary tumor, to study its heterogeneity. Shared SNP alterations, with stable allele fraction from germline to metastasis were detected, mainly affecting DNA repair genes and promoting genome instability. Early somatic alterations, shared by nevi and primary and metastatic tumors, included BRAFV600E and focal copy-loss of several genes, acquiring additional cancer hallmarks. Phylogenetic analyses revealed that these common somatic alterations would provide a "bridge", allowing progression from a benign to a malignant state. Distant and adjacent nevi were rich in alterations, presenting differential SNP and CNV alterations. Upon tumor transformation, a marked increase in CNV over SNP alterations was determined. Both the number of SNP and CNV-affected genes, including known driver genes, increased throughout progression, although TMB levels remained lower than expected for melanoma. Typical alterations in BRAFV600E tumors related to intrinsic resistance to targeted therapy were found, including BRAF amplification and loss of PTEN, CDKN2A/B, and TP53 surveillance genes. Finally, numerous metastatic alterations were detected, further promoting tumor progression. CONCLUSIONS: In this patient, longitudinal WES analysis revealed a sequential and cumulative pattern of genetic alterations, where germline and nevi somatic events contributed early to its rapid clinical progression. In this case report, we found tumor-associated nevi as genetically heterogeneous precursor entities, in which potential prognostic biomarkers should be studied prospectively.

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BACKGROUND: Laser hair removal (LHR) is a common practice with increasing use worldwide. Clinical and dermoscopic changes in melanocytic nevi after LHR have been reported but prospective studies are lacking. OBJECTIVE: To describe dermoscopic changes of melanocytic nevi at different time points after LHR. METHODS: Prospective study in a cohort of female patients undergoing diode LHR. Dermoscopic follow-up of at least three nevi on the legs that underwent hair removal. We included three nonexposed nevi on the arms as controls. Two blinded investigators analyzed dermoscopic images, according to variables selected based on the available literature. RESULTS: Thirty-four patients were included with a total of 148 nevi on the legs and 112 nevi on the arms (controls). 47.9% (71/148) of the nevi on the legs had evidence of dermoscopic changes at the sixth hair removal session, compared to 9.8% (11/112) on controls (p < 0.001). The most frequent change was "bleaching" (41.9%, 62/148). Also, we observed "irregular hyperpigmented areas," and "regression structures" in 5.4% (8/148) and 4.7% (7/148) of the cases at the sixth session, respectively. Neither of these structures were observed in the controls (p < 0.05). LIMITATIONS: Only females were included; we did not perform histopathological evaluation nor reflectance confocal microscopy of changing nevi. CONCLUSION: Melanocytic nevi frequently change after diode LHR. The changes cannot always distinguish between LHR induced and melanoma, so we advise avoiding nevi during laser therapies with melanin targets.

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Introducción: el nevus azul celular es una tumoración melanocítica dérmica benigna. En ocasiones, puede ser falsamente diagnosticada como lesiones malignas, entre ellas, el melanoma. Caso clínico: se trata de una mujer de 37 años que presentó una masa parotídea izquierda de cuatro meses de evolución correspondiente con un nevus azul celular. Discusión: la región de la cabeza y cuello es la tercera en frecuencia, tras la sacrococcígea y las extremidades. Ante una tumoración melanocítica, es importante la confirmación diagnóstica, debido a las similitudes, tanto clínicas como anatomopatológicas, del nevus azul celular con el melanoma maligno. Conclusiones: es muy importante el diagnóstico diferencial correcto, para lo cual es de ayuda el uso de las tinciones inmunohistoquímicas. El tratamiento de esta tumoración es la exéresis quirúrgica con márgenes, esto presenta un comportamiento benigno y baja tasa de recidiva.

Introduction: Cellular blue nevi is a benign dermal melanocytic tumor. Occasionally, it can be falsely diagnosed as malignant lesions, including melanoma. Clinical case: This is a 37-year-old woman who presented with a left parotid mass of four months of evolution, corresponding with a cellular blue nevi. Discussion: The region of the head and neck is the third in frequency, after the sacrococcygeal and the extremities. During the study of a melanocytic tumor, diagnostic confirmation with a biopsy is important, due to the similarities, both clinical and pathological, of cellular blue nevi with malignant melanoma. Conclusions: the correct differential diagnosis is very important, for which immunohistochemical study is helpful. The treatment of this tumor is the surgical excision with margins, presenting benign behaviour and low recurrence rate.

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Los nevus melanocíticos agminados (NMA) son poco reportados en la bibliografía mundial. El nevus agminado (NA), puede presentar varios orígenes, dependiendo de ello pueden desarrollar características displásicas, con riesgo potencial de desarrollar melanoma y entrar a formar parte del Síndrome de Nevus Displásico (SND) de acuerdo a su diagnóstico clínico, dermatoscópico, histológico e historia familiar. El objetivo del presente trabajo es presentar y discutir el caso clínico de un paciente masculino de 26 años de edad sin antecedentes patológicos, evaluado en la Clínica Dermatológica Skinlaser en Quito Ecuador en mayo 2020, que presentó múltiples nevus en la superficie corporal, especialmente en la espalda a nivel posterior e interescapular. El estudio enfatiza la importancia de los controles dermatoscópicos y el seguimiento para hacer el reconocimiento de signos de atipia y cambios que hacen sospechar de malignización(AU)

Agminate melanocytic nevus (AMN) are little reported in the world literature. The agminated nevus (NA) can have various origins, depending on it, they can develop dysplastic characteristics, with a potential risk of developing melanoma and become part of Dysplastic Nevus Syndrome (SND) according to its clinical, dermoscopic, histological and history diagnosis. family. The objective of this work is to present and discuss the clinical case of a 26-year-old male patient with no pathological history, evaluated at the Clinica Dermatologica Skinlaser in Quito Ecuador in May 2020, who presented multiple nevi on the body surface, especially in the back at posterior and interscapular level. The study emphasizes the importance of dermoscopic controls and follow-up are essential to recognize signs of atypia and changes that lead to suspicion of malignancy(AU)

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Introducción: El nevo melanocítico congénito es una lesión pigmentada melanocítica, que está generalmente presente en el momento del nacimiento. La dermatoscopia es una técnica útil en el diagnóstico de los nevos. Objetivo: Examinar las características clínicas y dermatoscópicas de pacientes pediátricos con nevos melanocíticos congénitos. Métodos: Investigación de tipo descriptivo transversal. La población incluyó 340 pacientes pediátricos que asistieron a la consulta de dermatoscopia del Hospital Pediátrico Universitario "José Luis Miranda", Santa Clara, entre abril 2016- abril de 2017. La muestra quedó constituida por 128 pacientes con diagnóstico de nevos melanocíticos congénitos. Los datos obtenidos se analizaron a través del paquete estadístico SPSS 21.0. Se emplearon los métodos de la estadística descriptiva. Resultados: El cambio clínico más frecuente fue el crecimiento en 76 (47,8 por ciento) nevos. La localización más comprometida fue en los miembros superiores con 28 (17,6 por ciento) nevos. Existió una relación estadísticamente significativa (p< 0,05) entre el tamaño de los nevos y la localización en zonas fotoexpuestas con la presencia de cambios clínicos. El patrón en empedrado (27,7 por ciento) fue el más frecuente; sin embargo, el patrón globular (24,5 por ciento) se observó en todas las localizaciones. Ninguno de los nevos detectados empeoró hacia el melanoma maligno. Conclusiones: La mayoría de los nevos melanocíticos congénitos en este trabajo aparecieron desde el nacimiento. La presencia de cambios clínicos fue más evidente en las regiones fotoexpuestas. No se observó ningún nevo con estructuras o patrones dermatoscópicos relacionados con malignidad(AU)

Introduction: Congenital melanocytic nevi is a melanocytic pigmented lesion, which is usually present at birth. Dermatoscopy is a useful technique in the diagnosis of nevi. Objective: To examine the clinical and dermatoscopic characteristics of pediatric patients with congenital melanocytic nevi. Methods: Cross-sectional descriptive type research. The data obtained were analyzed through the SPSS 21.0 statistical package. The methods of descriptive statistics were used. Results: The most common clinical change was growth, in 76 (47.8 percent) nevi. The most compromised location was in the upper members with 28 (17.6 percent) nevi. There was a statistically significant relation (p< 0.05) among the size of the nevi and the location in photoexposed areas with the presence of clinical changes. The cobbled pattern (27.7 percent) was the most common; however, the globular pattern (24.5 percent) was observed in all locations. None of the detected nevi worsen towards malignant melanoma. Conclusions: Most congenital melanocytic nevi are shown from birth. The presence of clinical changes was most evident in photoexposed regions. No nevi was observed with dermatoscopic structures or patterns related to malignancy(AU)

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SUMMARY: Inflammatory infiltrates are frequently present in melanocytic lesions, with different distribution and composition. Much attention has been devoted to tumor-infiltrating lymphocytes (TIL) in the tumor microenvironment, establishing their prognostic and predictive value in many malignancies, including melanoma. However, lymphocytes, albeit the most numerous and consistent presence, constitute only part of the immune microenvironment. Other inflammatory cells, including neutrophils, plasma cells, eosinophils and mast cells, are found in melanoma and other melanocytic lesions.Few studies offer a detailed count of these inflammatory infiltrates across the spectrum of melanocytic lesions. By using whole slide image analysis and open source software, in the present study we report the enumeration of different inflammatory infiltrates in benign melanocytic nevi, dysplastic nevi, melanoma in situ and invasive malignant melanomas. Significant higher numbers of plasma cells and neutrophils were observed in melanoma. These results indicate that composition of the inflammatory infiltrate may contribute to the diagnostic algorithm of melanocytic lesions.

RESUMEN: Los infiltrados inflamatorios están presentes con frecuencia en las lesiones melanocíticas, con diferente distribución y composición. Se ha prestado mucha atención a los linfocitos infiltrantes de tumores (TIL) en el microambiente tumoral, estableciendo su valor pronóstico y predictivo en muchas neoplasias malignas, incluido el melanoma. Sin embargo, los linfocitos de presencia más numerosa y constante, constituyen solo una parte del microambiente inmunológico. Otras células inflamatorias, incluidos neutrófilos, células plasmáticas, eosinófilos y mastocitos, se encuentran en el melanoma y otras lesiones melanocíticas. Pocos estudios ofrecen un recuento detallado de estos infiltrados inflamatorios en todo el espectro de lesiones melanocíticas. Mediante el uso de análisis de imágenes de diapositivas completas y software de código abierto, en el presente estudio informamos la enumeración de diferentes infiltrados inflamatorios en nevos melanocíticos benignos, nevos displásicos, melanoma in situ y melanomas malignos invasivos. Se observaron números significativamente más altos de células plasmáticas y neutrófilos en el melanoma. Estos resultados indican que la composición del infiltrado inflamatorio puede contribuir al algoritmo diagnóstico de las lesiones melanocíticas.

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Nevos melanocíticos são neoplasias benignas derivadas de melanócitos. O nevo melanocítico adquirido comum cutâneo é frequente na pele humana e apresenta maior incidência na terceira década de vida. E, embora uma taxa pequena de transformação maligna tenha sido estimada, considera-se que os nevos adquiridos sejam precursores de uma parcela dos melanomas cutâneos. A mutação somática BRAF p.V600E, que ativa a via MAPK/ERK e proliferação celular, está implicada na formação dos nevos adquiridos comuns de pele e de um grupo de melanomas cutâneos, de sítios não cronicamente expostos ao sol. A partir da caracterização molecular do melanoma seu tratamento foi aprimorado pelo uso de inibidores de Braf e Mek. O nevo melanocítico adquirido mucoso oral (NMO) e o melanoma mucoso oral (MMO) são lesões raras e de patogênese incerta. Há escassa literatura sobre aspectos moleculares do NMO e um número ligeiramente maior de estudos sobre os MMOs, em sua maioria em séries que englobam uma mistura de diferentes tipos de melanomas mucosos de diversos sítios. No presente estudo, investigou-se a mutação BRAF p.V600E em um grupo de 14 NMOs intramucosos e 7 MMOs primários, excluídas amostras de lábio, por meio de reação em cadeia da polimerase alelo-específico (PCR-AE). Realizou-se também uma revisão narrativa de literatura para calcular a frequência da mutação BRAF p.V600E em NMOs e MMOs. Foram incluídos artigos originais em língua inglesa que exibissem o sítio primário da lesão e status mutacional, seja por amostra ou sua frequência. Informações sobre a idade dos pacientes, país de origem e tipo de tumor, se primário, recorrente ou metastático, e técnica de análise do DNA utilizada também foram coletadas. Cinco das quatorze amostras de NMOs (35,7%) avaliadas no presente trabalho foram positivas para BRAF p.V600E, enquanto três das sete amostras de MMOs (42,8%) exibiram a mutação. Na revisão narrativa de literatura, em conjunto com nossos resultados, 19 NMOs foram avaliados e 8 NMOs apresentaram a mutação BRAF p.V600E, correspondendo a uma frequência de 42,1%. Dos 374 MMOs avaliados, 24 MMOs exibiram a mutação BRAF p.V600E, totalizando a frequência de 6,4%. Em conclusão, amostras de NMOs e MMOs foram analisadas quanto à presença da alteração genética oncogênica BRAF p.V600E. E junto à revisão da literatura pode- se calcular a frequência da mutação em NMOs e MMOs, contribuindo para uma melhor caracterização molecular dessas lesões.

Melanocytic nevi are benign neoplasms derived from melanocytes. Common cutaneous acquired melanocytic nevus is frequently in human skin and it has a higher incidence in the third decade of life. Although a low rate of malignant transformation is estimated, a portion of cutaneous melanoma is preceded by a melanocytic acquired nevus. BRAF p.V600E somatic mutation activates the MAPK/ERK pathway and cell proliferation. It is implicated in the cutaneous melanocytic acquired common nevus pathogenesis and cutaneous melanoma that arise in sites not chronically sun-exposed. After melanoma molecular description, its therapeutic was improved by Braf and Mek inhibitors. Oral mucosal acquired melanocytic nevus (NMO) and oral mucosal melanoma (MMO) are rare lesions with uncertain pathogenesis. There is scanty literature about NMOs molecular features and few studies on MMOs. Most articles are series that evaluate mucosal melanomas from several sites collectively. In the present study, BRAF p.V600E mutation was assessed in 14 intramucosal NMOs and 7 primary MMOs, excluding lip samples, by allele specific quantitative polymerase chain reaction (AS-qPCR). A narrative literature review had been performed to calculate BRAF p.V600E frequency in NMOs and MMOs. Original articles in English language were included, since it was possible to identify the primary sample site and mutational status, by sample or its frequency. Data about patient age, country, type of tumor (primary, recurrent or metastatic) and sequence technique used also were collected. Five in fourteen NMOs samples (35.7%) analyzed in the present study were BRAF p.V600E positive and three in seven MMOs samples (42.8%) showed the mutation. In the narrative literature review, added to our results, 19 NMOs were evaluated and 8 NMOs presented BRAF p.V600E mutation, corresponding to a frequency of 42.1%. Between 374 MMOs evaluated, 24 MMOs showed the mutation totalizing the frequency of 6.4%. In conclusion, BRAF p.V600E oncogenic mutation was assessed in NMOs and MMOs samples. Additionally, in combination with the literature review, it calculated the mutation frequency in NMOs and MMOs, improving the molecular characterization of those lesions.

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Introducción:Los Nevos Melanocíticos Congénitos (NMC) son lesiones cutáneas que frecuentemente están presentes desde el nacimiento, sin embargo,la presencia de un NMC gigante mayor a 20 cm es infrecuente, motivo de presentación del caso. Caso: Niño de 2 años y 5 meses, quien presentó Nevos congénitas de diferente diámetro dispersos en toda el área de la piel, siendo el más grande uno de color oscuro en el área de tórax posterior en línea media dorsal, abollonada que se eleva de la piel e inicia desde el occipucio y se prolonga por la línea media hasta llegar a la región sacra y glúteos, cubre hombros de forma triangular inversa con diámetros de 27 por 25 centímetros. Se acompañade numerosos nevos satelitales de 3 milímetros hasta 15 centímetros. La presencia de dos neurofibromasenlos dedos. Evolución: Una interconsulta a Neurología Pediátrica concluyó en un examen neurológico sin alteración, el estudio de Resonancia Magnética Nuclear Cerebral y del canal espinal,fueron normales, así como los exámenes complementarios de biometría hemática, química sanguínea, perfil hepático, perfil tiroideoy eco abdominal. La biopsia de piel reportó un patrón histológico de Nevo Melanocítico. Debido a la extensión de la lesión se decidió la observación. El prurito fue tratado sintomáticamente. Conclusión:El Síndrome del Nevo Melanocítico Congénito se asocia con múltiples hallazgos fenotípicos clásicos, dentro de los cuales se encuentran patrones de pigmentación que ocupan las líneas de Blaschko, neurofibromas y múltiples melanomas satélites. Su diagnóstico es clínico y para su tratamiento se requieren procedimientos quirúrgicos a consideración de la extensión de la lesión. El manejo integral de manera interdisciplinaria es fundamental en su tratamiento

Introduction: Congenital Melanocytic Nevi (CMN) are skin lesions that are frequently present from birth, however, the presence of a giant CMN greater than 20 cm is infrequent, reason for the presentation of the case. Case: A boy of 2 years and 5 months, who presented congenital nevi of different diameter scattered throughout the skin area, the largest being a dark-colored one in the posterior thorax area in the mid-dorsal line, embossed that rises from the skin and starts from the occiput and extends through the midline until it reaches the sacral region and buttocks, it covers shoulders in an inverse triangular shape with diameters of 27 by 25 centimeters. It is accompanied by numerous satellite nevi from 3 millimeters to 15 centimeters. The presence of two neurofibromas on the fingers. Evolution: A consultation with Pediatric Neurology concluded in a neurological examination without alteration, the study of Brain Nuclear Magnetic Resonance and of the spinal canal, were normal, as well as the complementary tests of hematic biometry, blood chemistry, liver profile, thyroid profile and abdominal echo . The skin biopsy reported a histological patternof Melanocytic Nevus. Due to the extent of the injury, observation was decided. The pruritus was treated symptomatically. Conclusion: Congenital Melanocytic Nevus Syndrome is associated with multiple classic phenotypic findings, among which are pigmentation patterns that occupy Blaschko's lines, neurofibromas and multiple satellite melanomas. Its diagnosis is clinical and its treatment requires surgical procedures, taking into account the extent of the lesion. Comprehensive management in an interdisciplinary manner is essential in its treatment

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Pacientes com nevo melanocítico congênito gigante possuem maior risco de desenvolver melanoma. Após o primeiro diagnóstico de melanoma, há também uma maior incidência de melanomas subsequentes em um mesmo paciente. No entanto, a terapêutica ideal para esta forma de nevo ainda é controversa. É relatado o caso de um paciente com nevo congênito gigante associado a melanoma múltiplo sincrônico e o tratamento proposto.

Patients with giant congenital melanocytic nevus are at higher risk of developing melanoma. After the first diagnosis of melanoma, there is also a higher incidence of subsequent melanomas in the same patient. However, the ideal therapy for this type of nevus is still controversial. We report the case of a patient with giant congenital nevus associated with multiple synchronous melanomas and the proposed treatment

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Abstract Spitz nevus is a benign melanocytic lesion, which presents in several ways: solitary, agminated, or disseminated. The disseminated variant is uncommon; it may have a rapid evolution (the eruptive form) and be difficult to manage. This report presents the case of a 24-year-old patient with multiple papules on his limbs, which had appeared four years previously. On physical examination, 120 pink and skin-colored papules were seen, which under dermoscopy were observed to be homogeneous, pink vascular lesions. Histopathologic study revealed epithelioid cells arranged in groups or singly in the dermis and dermo-epidermal junction. They were HMB-45 positive in the superficial dermis, and Ki-67 < 1%. Given these findings, a diagnosis of eruptive disseminated Spitz nevi was made.

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Spitz nevus is a benign melanocytic lesion, which presents in several ways: solitary, agminated, or disseminated. The disseminated variant is uncommon; it may have a rapid evolution (the eruptive form) and be difficult to manage. This report presents the case of a 24-year-old patient with multiple papules on his limbs, which had appeared four years previously. On physical examination, 120 pink and skin-colored papules were seen, which under dermoscopy were observed to be homogeneous, pink vascular lesions. Histopathologic study revealed epithelioid cells arranged in groups or singly in the dermis and dermo-epidermal junction. They were HMB-45 positive in the superficial dermis, and Ki-67<1%. Given these findings, a diagnosis of eruptive disseminated Spitz nevi was made.

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Acral lentiginous melanoma (ALM) is a rare subtype of melanoma with aggressive behavior. IMPDH enzyme, involved in de novo GTP biosynthesis, has been reported to assemble into large filamentary structures called rods/rings (RR) or cytoophidium (cellular snakes). RR assembly induces a hyperactive state in IMPDH, usually to supply a high demand for GTP nucleotides, such as in highly proliferative cells. We investigate whether aggressive melanoma tumor cells present IMPDH-based RR structures. Forty-five ALM paraffin-embedded tissue samples and 59 melanocytic nevi were probed with anti-IMPDH2 antibody. Both the rod- and ring-shaped RR could be observed, with higher frequency in ALM. ROC curve analyzing the proportions of RR-positive cells in ALM versus nevi yielded a 0.88 AUC. Using the cutoff of 5.5% RR-positive cells, there was a sensitivity of 80% and specificity of 85% for ALM diagnosis. In ALM, 36 (80%) showed RR frequency above the cutoff, being classified as RR-positive, compared with only 9 (15%) of the nevi (p < .001). Histopathology showed that 71% of the RR-positive specimens presented Breslow thickness > 4.0mm, compared with only 29% in the RR-low/negative (p = .039). We propose that screening for RR structures in biopsy specimens may be a valuable tool helping differentiate ALM from nevi and accessing tumor malignancy.

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Resumen Los nevos melanocíticos congénitos gigantes (NMCG) son lesiones melanocíticas secundarias a la migración anormal de los melanoblastos durante la embriogénesis. Afectan aproximadamente a 1 de cada 20,000 nacidos vivos y suelen estar presentes desde el nacimiento. Estas lesiones se distinguen porque cambian sus características morfológicas con el tiempo y aumentan su tamaño de forma paralela al crecimiento del niño, alcanzando un diámetro ≥ 20 cm en la edad adulta. La importancia de los NMCG radica en las complicaciones a las que se encuentran asociados, principalmente al desarrollo de melanoma o melanosis neurocutánea, además del impacto psicológico y social que generan en la mayoría de los casos, por lo que quienes los padecen requerirán de un seguimiento multidisciplinario a largo plazo. Actualmente, el manejo de los niños con NMCG continúa siendo controversial, ya que no existe un tratamiento de elección, por lo que este deberá ser individualizado de acuerdo con las características del nevo y las necesidades específicas de cada paciente.

Abstract Giant congenital melanocytic nevi (GCMN) are melanocytic lesions secondary to the abnormal migration of melanoblasts during the embryogenesis, affecting approximately one in 20,000 live births. They are usually present since birth and are distinguished by changing their morphological characteristics within time, and increasing their size parallel to the growth of the child, reaching a diameter ≥ 20 cm in adulthood. The importance of the GCMN lies in the complications associated to them; mainly the development of melanoma or neurocutaneous melanosis, in addition to the psychological or social impact that generates in most of the cases. Therefore, individuals with GCMN will require a multidisciplinary long-term follow-up. Currently, the management of children with GCMN is still controversial since there is no treatment of choice. Consequently, the treatment must be individualized according to the characteristics of the nevus and the specific needs of each patient.

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Resumen: Los nevos melanocíticos congénitos son una proliferación melanocítica benigna presente al nacimiento o que surgen en los primeros 2 o 3 años de vida. Habitualmente se clasifican, según su tamaño, en pequeños, medianos y grandes. Su importancia radica en el potencial riesgo de desarrollar melanoma, en la repercusión que tienen en la calidad de vida de quien los padece y en la asociación con disrafismo y tumores del sistema nervioso central. A mayor tamaño, mayor riesgo de desarrollar melanoma en el nevo o fuera de él. Describiremos las características epidemiológicas, clínicas dermatoscópicas y revisaremos el manejo y seguimiento de los nevos congénitos.

Summary: Congenital melanocytic nevi are a benign melanocytic proliferation present either from birth or during the first 2 or 3 years of life. They are usually classified according to size as: small, medium and large. Their importance lies on the potential risk of developing melanoma, on the impact they have on the patient's quality of life and on its association with dysraphism and tumors of the central nervous system. The larger the size of the nevi, the higher the risk of developing melanoma inside or outside the nevus. We will describe the epidemiological and dermatoscopic clinical characteristics and review the management and follow-up of congenital nevi.

Resumo: Os nevos melanocíticos congênitos são uma proliferação melanocítica benigna presente desde o nascimento ou durante os primeiros 2 ou 3 anos de vida. Eles são geralmente classificados de acordo com o seu tamanho como: pequenos, médios ou grandes. Sua importância está no risco potencial de desenvolver melanoma, no impacto que eles têm na qualidade de vida do paciente e na sua associação com disrafismo e tumores do sistema nervoso central. Quanto maior o tamanho dos nevos, maior o risco de desenvolver melanoma dentro ou fora do nevo. Descreveremos as características clínicas epidemiológicas e dermatoscópicas dos nevos congênitos e revisaremos o seu gerenciamento e acompanhamento.

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Giant congenital melanocytic nevi (GCMN) are melanocytic lesions secondary to the abnormal migration of melanoblasts during the embryogenesis, affecting approximately one in 20,000 live births. They are usually present since birth and are distinguished by changing their morphological characteristics within time, and increasing their size parallel to the growth of the child, reaching a diameter ≥ 20 cm in adulthood. The importance of the GCMN lies in the complications associated to them; mainly the development of melanoma or neurocutaneous melanosis, in addition to the psychological or social impact that generates in most of the cases. Therefore, individuals with GCMN will require a multidisciplinary long-term follow-up. Currently, the management of children with GCMN is still controversial since there is no treatment of choice. Consequently, the treatment must be individualized according to the characteristics of the nevus and the specific needs of each patient.

Los nevos melanocíticos congénitos gigantes (NMCG) son lesiones melanocíticas secundarias a la migración anormal de los melanoblastos durante la embriogénesis. Afectan aproximadamente a 1 de cada 20,000 nacidos vivos y suelen estar presentes desde el nacimiento. Estas lesiones se distinguen porque cambian sus características morfológicas con el tiempo y aumentan su tamaño de forma paralela al crecimiento del niño, alcanzando un diámetro ≥ 20 cm en la edad adulta. La importancia de los NMCG radica en las complicaciones a las que se encuentran asociados, principalmente al desarrollo de melanoma o melanosis neurocutánea, además del impacto psicológico y social que generan en la mayoría de los casos, por lo que quienes los padecen requerirán de un seguimiento multidisciplinario a largo plazo. Actualmente, el manejo de los niños con NMCG continúa siendo controversial, ya que no existe un tratamiento de elección, por lo que este deberá ser individualizado de acuerdo con las características del nevo y las necesidades específicas de cada paciente.

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BACKGROUND: Melanocytic nevi can vary in size and number in pregnant women, and the differential diagnosis with melanoma may be challenging. OBJECTIVES: To describe changes in total body photography of pregnant women and dermoscopy aspects of their nevi. METHODS: A prospective cohort study with 703 melanocytic nevi from 18 women was performed, comparing them in the first and third trimester of pregnancy. Images were analyzed between the 2 periods for changes in dermoscopic aspects. RESULTS: Total body photography images indicated that 44% of patients had new lesions. Regarding the observed changes, there were symmetric or regular changes of the network (23% of cases), occurrence of new globules/dots (12.4%), new vascular structures (3.2%), new streaks (1.7%), and new structureless area (1.0%). Moreover, 55.0% of the nevi increased in size. Enlarging of the nevi was observed mostly on the abdomen (87.1%; P < 0.001) and showed more network changes (27.1%; P = 0.014) and formation of new globules and dots (16.0%; P < 0.001). Patients with a risk of developing melanoma presented more frequently enlarged nevi (45%; P = 0.019). The association between streak formation and skin type was significant (P = 0.012) and was more frequent in skin type II (2.7%), when compared with skin types III (1.3%) and IV (0%). CONCLUSIONS: Development of new melanocytic nevi may occur in pregnant women. The majority of the preexisting melanocytic nevi showed enlarging, and most of them presented with benign dermoscopic changes. The appearance of new streaks is more frequent in fair skin types. Patients with a personal or family history of melanoma in first-degree relatives presented more nevi with changes in size.