Total body mapping in the follow-up of melanocytic lesions: recommendations of the Brazilian Society of Dermatology

Abstract Total body mapping comprises photographic documentation of the entire body surface followed by digital dermatoscopy of selected melanocytic lesions, aiming to compare their evolution over time and identify new lesions. As this is an exam based on comparative analysis of serial dermoscopic body images, standardization of the technique for performing total body mapping is essential. Prepared by specialists from the Brazilian Society of Dermatology, using the modified Delphi method, this article provides recommendations for carrying out total body mapping in Brazil, regarding its indications, technical aspects, and the issuing of the report.

Dermatoscopía de nevos melanocíticos congénitos acrales: puesta al día / Dermoscopy of melanocytic nevi acral congenitals: update

Los nevos melanocíticos congénitos (NMC) son aquellos presentes al nacer o que se desarrollan durante los primeros dos años de vida. Hay muy pocos casos documentados de melanoma lentiginoso acral en asociación con nevos preexistentes en comparación a otros subtipos de melanoma. De éstos, la mayoría serían asociados a nevos melanocíticos adquiridos acrales (NMAA) y muy excepcionalmente asociados a nevos melanocíticos congénitos acrales (NMCA). Sin embargo, la extirpación de lesiones pigmentadas acrales congénitas es practicada con frecuencia y se desconocen los patrones dermatoscópicos más característicos, así como tampoco se disponen de algoritmos de seguimiento. A continuación, presentamos dos casos de NMCA con patrones dermatoscópicos característicos y realizamos una revisión de los patrones dermatoscópicos más comunes de NMCA descritos en la literatura.

Congenital melanocytic nevi (CMN) are those present at birth or that develop during the first two years of life. There are very few documented cases of acral lentiginous melanoma in association with pre-existing nevi compared to other subtypes of melanoma. Of these, the majority would be associated with acral acquired melanocytic nevi (NMAA) and very exceptionally associated with acral congenital melanocytic nevi (NMCA). However, the excision of congenital acral pigmented lesions is frequently practiced and the most characteristic dermoscopic patterns are unknown, nor are there any follow-up algorithms. In the following, we present two cases of NMCA with characteristic dermoscopic patterns and a review of the most common dermoscopic patterns of NMCA described in the literature

Histogenetic categorization of atypical melanocytic tumor of uncertain biological malignant potential

Abstract: Several reports have demonstrated difficulties and lack of agreement in the histopathological diagnosis of particular melanocytic lesions, with problems in their management. A histogenetic approach to the study of these lesions originated the following classification: 1. superficial atypical proliferation significance; 2. melanocytic tumor of uncertain potential; 3. pigmented epithelioid melanocitoma of uncertain potential; 4. microinvasive radial growth phase of uncertain potential. The terminology remains controversial, reflecting the uncertainty of the diagnosis and the biological potential of these atypical melanocytic lesions.

A avaliação dos nevos comuns, nevos atípicos e melanomas iniciais (lesões melanocíticas duvidosas) através da microscopia confocal in vivo / The evaluation of common nevi, atypical nevi and early melanomas (doubtful melanocytic lesions) by in vivo confocal microscopy

Introdução: O diagnóstico clínico, dermatoscópico e histopatológico dos nevos atípicos é um desafio, uma vez que podem apresentar padrões regulares, observados em nevos melanocíticos comuns, ou padrões irregulares e assimétricos, que se assemelham aos padrões encontrados em melanomas e assim persiste a necessidade de novas técnicas de imagem não invasivas, como microscopia confocal in vivo, para se melhorar a acurácia diagnóstica dessas lesões. Objetivos: Descrever as características. encontradas no exame de microscopia confocal in vivo nas lesões melanocíticas, correlacioná-las com seus diagnósticos histopatológicos,elaborar e validar um algoritmo com as características observadas para diferenciar as lesões melanocíticas entre si. Material e Método: Trata-se de um estudo observacional realizado no Departamento de Oncologia Cutânea do A.C.Camargo Cancer Center. Foram incluídas 110 lesões suspeitas pela análise clínica e dermatoscópica de nevo atípico. Todas as lesões foram analisadas com exame de microscopia confocal e posterior exame histopatológico. Os dados obtidos foram compilados para análise estatística, a fim de estabelecer um algoritmo. Foi realizada a regressão logística simples e regressão logística múltipla em três grupos: 1) comparação do nevo melanocítico comum versus nevo melanocítico atípico; 2) comparação do nevo melanocítico atípico versus melanoma e 3) comparação do nevo melanocítico comum e atípico versus melanoma. Com os dados obtidos na regressão logística múltipla e identificação dos critérios estatisticamente. significantes, foi criado um algoritmo e um nomograma para ajudar a diferenciar as lesões melanocíticas, com base nos critérios da microscopia confocal in vivo. Para validar esse algoritmo, 30 novos casos foram analisados por três examinadores experientes. Resultados: A análise através da regressão logística simples e múltipla mostrou que no grupo 3 é possível diferenciar estatisticamente os nevos comuns e atípicos dos melanomas com base na presença de três critérios: células redondas na. JDE (p = 0,048), hotspot periférico na JDE (p = 0,032) e "sheet of cells" (p = 0,04). Essas mesmas três características foram usadas como critérios na elaboração de um algoritmo através de um nomograma cuja finalidade é indicar a probabilidade de uma dada lesão ser um melanoma. A validação desse algoritmo foi realizada em 30 novos casos por três examinadores independentes, os quais apresentaram resultados semelhantes com alta sensibilidade e especificidade: 100% sensibilidade, 83,3% especificidade e acurácia de 86,67%. Esses valores foram obtidos através da curva ROC. onde o ponto de corte de 158 pontos confere uma probabilidade estimada de 70% para o diagnóstico do melanoma. Conclusão: Ao correlacionar as características encontradas no exame microscopia confocal in vivo de cada lesão com seu diagnóstico histopatológico final, foi observado que essas características (células redondas na JDE, hotspot na JDE de localização periférica e sheet of cells) apresentavam prevalências distintas e estatisticamente significantes entre as diferentes lesões melanocíticas, que foram usadas e validadas como critérios em um algoritmo para diferenciar nevos comuns e atípicos dos melanomas

Introduction: Clinical, dermatoscopic and histopathological diagnosis of atypical nevi is a challenge, because they may present regular patterns, observed in common melanocytic nevi, or irregular and asymmetric patterns, which resemble the patterns found in melanomas and thus still existing the need for development of new non-invasive imaging techniques such as in vivo microscopy confocal, in order to improve the diagnostic accuracy of these lesions. Objectives: Describe the characteristics found at in vivo confocal microscopic examination of melanocytic lesions and correlate them with their histopathological diagnoses. Also, elaborate and validate an algorithm with the characteristics observed to differentiate this lesions. Material and Method: This observational study was conducted at the Cutaneous Oncology Department of the A.C.Camargo Cancer Center. 110 suspicious lesions were included as atypical nevi after clinical and dermaoscopy examination. All lesions were later analyzed with confocal microscopy and histopathological examination. All data obtained were compiled for statistical analysis in order to establish an algorithm to aid the diagnosis of atypical nevi and other melanocytic lesions. Comparisons by simple and multiple logistic regression were performed in three groups: 1) common melanocytic nevi versus atypical melanocytic nevi; 2) atypical melanocytic nevi versus melanoma; and 3) common and atypical melanocytic nevi versus melanoma. With data obtained from multiple logistic regression and identification of the statistically significant criteria, an algorithm and a nomogram were created to help differentiate the melanocytic lesions based on in vivo confocal microscopy findings. To validate this algorithm, 30 new cases were analyzed by three experienced examiners. Results: Differentiation of common and atypical nevi from melanoma was statistically possible based on the presence of three criteria: round cells at the DEJ (p = 0.048), peripheral hotspot at the DEJ (p = 0.032) and sheet of cells (p = 0.04). These same three features were used as criteria to elaborate an algorithm and a nomogram capable to indicate the probability of a given lesion being a melanoma. The validation of this algorithm was performed by three independent examiners, who presented similar results with high sensitivity and specificity: 100% sensitivity, 83.3% specificity and 86.67% accuracy. These values were obtained through the ROC curve where the cut-off point of 158 points is related to 70% estimated probability for melanoma diagnosis. Conclusion: The correlation of in vivo confocal microscopy features found in each lesion with its final histopathological diagnosis revealed that round cells at DEJ, hotspot peripheral location at DEJ and sheet of cells had statistically significant different prevalence between different melanocytic lesions, and these features were used and validated as criteria in an algorithm to differentiate common and atypical nevi from melanomas

Síndrome de nevo de Becker con características ictiosiformes / Becker's nevus syndrome with ichthyosiformes features

El nevo de Becker, es un hamartoma cutáneo benigno de etiología desconocida, que se presenta como una mácula hiperpigmentada de bordes geográficos. Por lo general, se ubica en la región torácica superior y su compromiso tiende a ser unilateral. Ocasionalmente el nevo de Becker se asocia a anomalías en el tejido óseo, muscular o cutáneo, siendo la ictiosis una característica muy infrecuente. La presencia de alguna de estas anomalías asociadas a un Nevo de Becker determina el síndrome del Nevo de Becker. Paciente de sexo masculino de 18 años, que consultó por un cuadro iniciado en la infancia, caracterizado por la aparición de manchas cafés, escamosas, pruriginosas que inicialmente se ubicaron en las extremidades inferiores y con el tiempo fueron apareciendo en tronco, región lumbar y glútea. Mediante la correlación clínico-patológica se llegó al diagnóstico de Nevo de Becker, con características ictiosiformes. Se realizaron estudios complementarios con ecocardiograma Doppler y radiografía de tórax, que resultaron normales y una ecotomografía Doppler color de tórax anterior que mostró una leve ginecomastia bilateral con discreto aumento del botón mamario derecho y una leve hipoplasia del pectoral mayor derecho diagnosticándose Síndrome de Nevo de Becker. La mayoría de los reportes de Síndrome de nevo de Becker describen anomalías como escoliosis o hipoplasia unilateral de la mama, con escasos reportes sobre ictiosis. Se reporta este caso por su presentación atípica con múltiples nevos de Becker, compromiso de hemicuerpo inferior y su asociación ictiosiforme poco descrita en la literatura.

Becker's nevus is a benign cutaneous hamartoma of unknown etiology; it appears as a hyperpigmented macula with geographical borders. Usually it is located in the upper thoracic region, unilaterally. Occasionally Becker nevus is associated with abnormalities in the bony, muscle or skin tissue, being ichthyosis an unusual feature. The presence of some of these anomalies associated with Becker´s nevus determine the Becker´s nevus syndrome. Male patient of 18 years old who had a clinical history that begun in childhood, characterized by the appearance of brown, scaly, itchy patch that initially were located in the lower extremities and eventually were appearing in the trunk, lumbar and gluteal area. The clinical-pathological correlation led us to the diagnosis of Becker´s nevus with ichthyosiform features. Additional studies were performed such as Doppler echocardiography and chest radiograph, both normal. Color Doppler ultrasonography of anterior chest showed a slight bilateral gynecomastia with discrete increase of the right breast and a slight hypoplasia of the right major pectoral, determining a Becker´s nevus syndrome. Most reports of Becker´s nevus syndrome described abnormalities such as scoliosis or unilateral breast hypoplasia, few cases have been reported with ichthyosis. This case is reported for its atypical presentation with multiple Becker´s nevus, compromise of the lower body and its ichthyosiform association, rarely described in the literature.

Diagnóstico e conduta em melanocitoma do disco óptico / Diagnosis and management of optic disc melanocytoma

RESUMO O melanocitoma do disco óptico é um tumor benigno, com pouca predisposição a transformação maligna para melanoma. Geralmente é assintomático, contudo deve ser avaliado periodicamente com exames, sendo um deles a campimetria computadorizada que se encontra alterada em 90% dos casos. Relatamos o caso de um homem de 61 anos sem diagnóstico prévio de melanocitoma do disco óptico. Discute-se a importância de um diagnóstico diferencial com melanoma e acompanhamento seriado do paciente.

ABSTRACT The optic disc melanocytoma is a benign tumor, with little predisposition to become a melanoma. It is usually asymptomatic, nonetheless should be evaluated periodically, most often by computerized perimetry, which in 9 out of 10 cases will show some alteration. In the following paper we report the case of 61 years old man without previous diagnosis of optic disc melanocytoma and discuss the importance of melanoma differential diagnosis and monitoring.

Identification of Somatic KRAS Mutation in a Korean Baby with Nevus Sebaceus Syndrome

No abstract available.

Nevus melanocítico congénito, a propósito de un caso / Congenital melanocytic nevi, case report

INTRODUCCIÓN: Los nevus melanocíticos son proliferaciones benignas de células névicas. Los nevus melanocíticos congénitos (NMC) representan el 1 por ciento del total y según su tamaño se clasifican en pequeños, medianos o gigantes. PRESENTACIÓN DEL CASO: Paciente de sexo masculino, 6 años de edad, con NMC gigante en forma de traje de baño. Controlado en policlínico de Dermatología desde su nacimiento, se mantuvo en observación realizándose exámenes imagenológicos, biopsias de piel y nódulos. En reunión multidisciplinaria respecto al caso, se plantea tratamiento quirúrgico incisional por etapas. DISCUSIÓN: El manejo de los NMC es controversial, se describen múltiples terapias que incluyen escisión, dermoabrasión, ablación con láser, etc. Pero para disminuir el riesgo de malignización la única efectiva es la escisión, no siempre posible en los NMC gigantes. Para los NMC de pequeño o mediano tamaño se recomienda un manejo individualizado con evaluaciones periódicas con dermatoscopía. En el caso de los NMC gigantes la mayoría de los autores concuerda en una extirpación temprana agresiva para disminuir el riesgo de malignización. En el caso clínico expuesto, el paciente presenta factores de riesgo para las dos principales complicaciones, por lo que se plantea el tratamiento quirúrgico. La remoción completa frecuentemente necesita de escisión por partes, usando expansores de piel e injertos dérmicos. Independiente de la terapia que se elija hay que considerar la necesidad del apoyo psicológico en este tipo de lesiones.

INTRODUCTION: Melanocytic nevi are benign proliferations of nevus cells. Represent 1 percent of all melanocytic nevi and are classified by size into small, medium or giant. CASE REPORT: Male patient, 6-year-old with giant congenital melanocytic nevi (CMN) as swimsuit. Controlled in the Department of Dermatology at birth, was kept under observation imaging tests, and skin biopsies performed nodules. In multidisciplinary meeting on the case, incisional surgical treatment arises in stages. DISCUSSION: The management of NMC is controversial; multiple therapies are described, including excision, dermabrasion, laser ablation, etc. But excision is the only way to reduce the risk of malignancy, not always possible in the giant NMC. NMC for small to medium size individualized management with periodic evaluations with dermoscopy is recommended. In the case of the giant NMC most authors agree on an aggressive early removal to reduce the risk of malignancy. In the case report, the patient had risk factors for the two major complications, so that surgical treatment is considered. Complete removal often requires cleavage by parts, using skin expanders and skin grafts. Independent of therapy you choose must consider the need for psychological support in this type of injury.

A case of collision tumor or transdifferentiation between malignant melanoma and leiomyosarcoma.

A 73-year-old woman was referred to the hospital due to a pigmented, asymptomatic nevus on her right arm that had changed in size and color. The histopathological examination showed a superficial spreading malignant melanoma, Clark level III, 2.26 mm in thickness. Two years later, the patient presented a 10 cm rapidly growing mass in her right axilla. The mass in the axilla measured 12.5 ΄ 9 ΄ cm. It revealed a lymph node metastases with a tumor growth composed of two different contiguous morphological and immunohistochemical components, respectively, melanosomes and leiomyosarcoma. The combination of a melanocytic nevus with other tumor of epidermal or adnexal origin has been described before, but still the co-existence of two different neoplasms within a lesion is still uncommon. The most common combination is basal cell carcinoma and melanocytic nevus or one of them together with a seborrheic keratosis. There have also been occasional reports of rhabdomyosarcomatous differentiation. However, mesenchymal differentiation, and in this case leiomysarcoma, with formation of heterologous elements in melanocytic tumor is very rare. Another plausible explanation may be that malignant melanoma cells could have transdifferentiated into a leiomyosarcomatoid phenotype with resulting metastases of either type. Malignant melanomas have shown a wide variety of cytological changes and can mimic carcinomas, lymphomas, and sarcomas. Spindle cell melanomas commonly simulate spindle cell carcinomas. It has also been documented that desmoplastic melanomas can change into fibroblastic, Schwannian, and myofibroblastic differentiation.

Phacomatosis Pigmentokeratotica without Extracutaneous Abnormalities: A Case Study Involving a Preterm Baby

Phacomatosis pigmentokeratotica (PPK) is a rare syndrome defined by the association of an organoid nevus occasionally with sebaceous differentiation, a speckled lentiginous nevus, and other extracutaneous anomalies. A preterm male infant of only 830 g at 27 week gestational age had an organoid nevus showing sebaceous differentiation. Also, he had multiple speckled-lentiginous nevus. Correlating the observed clinical presentation with the histopathological findings, the diagnosis of PPK was established. There have been less than 10 cases of PPK without extracutaneous manifestation. We present an uncommon case of a preterm patient with PPK who had no extracutaneous abnormalities.

Nevo da epidermólise bolhosa: caso clínico e revisão da literatura / Epidermolysis bullosa nevus: case report and literature review

Lesões melanocíticas adquiridas assemelhando-se à melanoma têm sido descritas nos principais grupos da Epidermólise bolhosa, e referidas como "Nevos da Epidermólise bolhosa''. Induzem facilmente ao erro diagnóstico, apesar de nenhuma transformação maligna ter sido descrita. Relatamos o desenvolvimento de um nevo melanocítico adquirido grande no local de bolhas recorrentes em uma criança de 5 anos portadora de Epidermólise bolhosa simples. O padrão dermatoscópico global foi sugestivo de benignidade, e os achados histopatológicos foram compatíveis com um nevo melanocítico composto. Este é o primeiro caso de um Nevo da Epidermólise bolhosa publicado na literatura brasileira.

Acquired melanocytic lesions resembling malignant melanoma have been described in all major categories of Epidermolysis bullosa and referred to as "Epidermolysis bullosa nevi''. They easily induce to diagnostic error, although no malignant transformation has been reported. We report the development of a large acquired melanocytic nevus at a site of recurrent blisters in a 5-year-old child with Epidermolysis bullosa simplex. The global dermoscopic pattern was suggestive of benignity, and the histopathological findings were compatible with a compound melanocytic nevus. This is the first published case of Epidermolysis bullosa nevi in Brazilian literature. Despite their benign behavior, we emphasize the importance of regular clinical and dermoscopic monitoring, since a malignant course still cannot be totally excluded.

Deep penetrating nevus: A distinct variant of melanocytic nevus.

Deep penetrating nevus (DPN) is a variant of melanocytic nevus which goes unrecognized due to its relative rarity and may be misinterpreted as malignant melanoma. It commonly presents in young adults as a dark pigmented lesion on the face, neck, or shoulder. A 60-year-old lady presented with a mole over the left arm of 8 years duration. A biopsy of the lesion was performed under the clinical impression of a compound nevus with suspicion of malignancy. Based on the histologic features, a diagnosis of DPN was put forward.

Tumores en colisión: queratosis seborreica con nevus melanocítico. Estudio dermatoscópico e histopatológico a propósito de dos lesiones / Collision tumours: seborrheic keratosis and melanocytic nevus. Dermoscopy and histopathology of two lesions

Los tumores en colisión son raros, y representan la asociación de más de una neoplasia en un mismo preparado histológico y separados por menos de 1 mm. Aunque los nevus melanocíticos y las queratosis seborreicas son tumores frecuentes, hay pocos casos publicados de esta asociación. Comunicamos el de un hombre de 54 años, con historia de síndrome de nevus displásico y melanoma, que consultó por dos lesiones pigmentadas con cambios recientes. El caso fue estudiadocon dermatoscopia e histopatología.

Halo nevo: nevo de Meyerson con rasgos de nevo de Sutton / Halo nevo: Meyerson´s nevus with Sutton´s nevus aspects

En 1971 Meyerson describió un halo inflamatorio alrededor de un nevo melanocitico. Este tipo de nevo fue denominado nevo de Meyerson. El halo nevo de Sutton, también denominado leucodermia centrífuga akquirida, fue descripto en 1916 y se caracteriza por hipomelanosis que rodea a un nevo melanocítico. Se comunica el caso de una paciente con un nevo melanocítico con halo eccematoso (nevo de Meyerson) cuyo patrón histológico combina caracteres de nevo de Sutton con nevo de Meyerson.