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This case report explores the progression of cutaneous lymphoid dyscrasia to a lymphoma in a 13-year-old boy with pityriasis lichenoides chronica and lichenoid pigmented purpuric dermatitis, the latter evolving into mycosis fungoides. We highlight some of the crucial clinical and histological characteristics to differentiate between these entities. Early identification of this progression is critical not only for preventing further lymphoma development but also for initiating prompt therapy.
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Introduction: The umbilicus is a unique physiologic scar of human body. Its absence leads to an unnatural abdominal appearance and can cause psychological and social complexes. Objective: To present a case of neoumbilicoplasty with vertical island pedicle flap for a large pigmented umbilical nevus. Clinical case: A healthy 50-year-old female patient with a history of a pigmentated nevus on the umbilicus since childhood that complains of changes in size and color of it. She also referred notice a lump in the upper umbilical region which was an umbilical hernia. The patient underwent an omphalectomy with oncological margins, an umbilical hernia repair and a neoumbilicoplasty with vertical island pedicle flap. Seroma was the only postoperative complication. Aesthetic results were acceptable and satisfied the patient. Conclusions: This is a rare case not only because the umbilical skin lesions are uncommon but the neoumbilicoplasty technique is rarely used(AU)
Introducción: El ombligo es la única cicatriz fisiológica del cuerpo humano. Su ausencia provoca un aspecto abdominal antinatural y puede provocar complejos psicológicos y sociales. Objetivo: Presentar un caso de neoumbilicoplastia con colgajo pediculado vertical en isla para un nevo umbilical pigmentado de gran tamaño. Caso clínico: Paciente femenina sana de 50 años con antecedentes de nevo pigmentado en ombligo desde la infancia, que refiere cambios en el tamaño y color de este. También refirió notar un bulto en la región umbilical superior que era una hernia umbilical. La paciente fue sometida a onfalectomía con márgenes oncológicos, reparación de la hernia umbilical y neoumbilicoplastia con colgajo pediculado vertical en isla. El seroma fue la única complicación postoperatoria. Los resultados estéticos fueron aceptables y satisficieron a la paciente. Conclusiones: Es un caso raro, no solo porque las lesiones cutáneas umbilicales son poco comunes, sino que la técnica de neoumbilicoplastia, rara vez se utiliza(AU)
الموضوعات
Humans , Female , Middle Aged , Umbilicus/surgery , Plastic Surgery Procedures/methods , Dermoscopy/methods , Hernia, Umbilical/diagnosis , Nevus, Pigmented/surgery , Seroma/complicationsالملخص
Resumen La incontinencia pigmenti(IP) es una genodermatosis infrecuente ysistémica del neuroectodermo que involucra la piel, el sistema nervioso central, los ojos y los dientes, entre otros. Los signos clínicos dermatológicos constituyen el principal criterio diagnóstico, debido a que suelen ser los primeros en manifestarse. Se describen cuatro estadios característicos de la enfermedad según las lesiones cutáneas predominantes. No obstante, su pronóstico depende de los signos y síntomas extracutáneos. El diagnóstico se centra en criterios clínicos, histopatológicos y/o genéticos. Visto que no existe una terapéutica específica, la atención médica de esta enfermedad es multidisciplinaria y sintomática, y debe acompañarse del asesoramiento genético a los afectados y sus familiares. Presentamos el caso de una niña, nacida a término completo, quien presentó un cuadro clínico compatible con incontinencia pigmenti, del cual detallamos su progresión clínica, diagnóstico y seguimiento.
Abstract Incontinentiapigmenti is a rare and multisystemic,neuroectodermal genodermatosis that involves the skin, central nervous system, eyes and teeth, among others. Dermatological clinical signs are the main diagnostic criteria because they are usually the first to manifest. Four characteristic stages of the disease are described according to the predominant skin lesions. Nonetheless, prognosis depends on extracutaneous clinical signs and symptoms. Diagnosis is based on clinical, histopathological and/or genetic criteria. Considering there is no specific treatment available, the management of this disease is multidisciplinary and symptomatic, and must be accompanied by genetic counseling for those affected and their families. We present in a full-tern newborn femalethat presented with a clinical picture compatible with incontinentiapigmenti, and we will detail the clinical progression, diagnosis, and follow-up.
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Abstract Background: A lot of congenital melanocytic nevi (CMN) carry the somatic mutation in the oncogene BRAF V600E. But the detailed histopathologic characteristics and the proliferative activity of CMN with BRAF V600E gene mutation have not been systematically documented. Objective: To identify the proliferative activity and histopathological features correlating them with BRAF V600E gene mutation status in CMN. Methods: CMN were retrospectively identified from the laboratory reporting system. Mutations were determined by Sanger sequencing. The CMN were divided into a mutant group and control group according to whether there was BRAF gene mutation and were strictly matched according to gender, age, nevus size, and location. Histopathological analysis, analysis of Ki67 expression by immunohistochemistry and laser confocal fluorescence microscopy were performed. Results: The differences in Ki67 index, the depth of nevus cell involvement and the number of nevus cell nests between the mutant group and the control group was statistically significant, with p-values of 0.041, 0.002 and 0.007, respectively. Compared with BRAFV600E negative nevi, BRAF V600E positive nevi often exhibited predominantly nested intraepidermal melanocytes, and larger junctional nests, but the difference in this datasets were not statistically significant. The number of nests (p = 0.001) was positively correlated with the proportion of Ki67 positive cells. Study limitations: A small sample of patients were included and there was no follow-up. Conclusions: BRAF V600E gene mutations were associated with high proliferative activity and distinct histopathological features in congenital melanocytic nevi.
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Bee stings usually result in mild allergic reactions; however, mass envenomation can cause severe complications such as rhabdomyolysis, hemolysis, shock, or multi-organ damage. Rhabdomyolysis can result in acute renal failure either by tubular obstruction by myoglobin casts or by direct cytotoxic injury. We present a case of a 12-year-old female child who presented with sudden onset anuria and hypertension following mass envenomation by bees. A renal biopsy was performed, the microscopic evaluation of which revealed tubular injury, with associated intratubular pigmented casts. The casts stained positive for myoglobin immunohistochemical stain, thus confirming a diagnosis of myoglobin cast nephropathy. The patient was given IV steroids and underwent seven sessions of hemodialysis, following which there was complete recovery of renal function.
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Abstract Background: Pigmented (or melanocytic) neurofibroma (PN) constitutes only 1% of cases and is considered a rare variant of neurofibroma containing melanin-producing cells. In addition, the association of PN with hypertrichosis is infrequent. Case report: We describe the case of an 8-year-old male with a neurofibromatosis type 1 (NF1) diagnosis, who presented a light brown hyperpigmented plaque, smooth and well-demarcated, and hypertrichosis on the left thigh. The skin biopsy showed characteristics of neurofibroma; however, in the deep portion of the lesion, melanin deposits positive for S100, Melan-A, and HMB45 were observed, thus establishing the diagnosis of pigmented neurofibroma. Conclusions: Although PN is a rare subtype of neurofibroma, it is considered a chronically progressive benign tumor containing melanin-producing cells. These lesions can appear alone or in association with neurofibromatosis. Since this is a tumor that can be confused with other skin lesions, biopsy analysis is essential to differentiate it from other pigmented skin tumors, such as melanocytic schwannoma, dermatofibrosarcoma protuberans, neurocristic hamartoma, or neuronevus. Surveillance is part of the treatment, and surgical resection is sometimes performed.
Resumen Introducción: El neurofibroma pigmentado (NP) o melanocítico constituye solamente el 1% de los casos y se considera como una variante rara del neurofibroma que contiene células productoras de melanina. Además, la asociación de NP con hipertricosis es muy rara. Caso clínico: Se describe el caso de un paciente de sexo masculino de 8 años 2 meses de edad con diagnóstico de neurofibromatosis tipo 1 (NF1), quien presentaba en la cara anterior del muslo izquierdo una placa hiperpigmentada de color café claro, bien delimitada y de consistencia suave, e hipertricosis. La biopsia de piel presentó cambios característicos de neurofibroma; sin embargo, en la porción profunda de la lesión se observaron depósitos de melanina positivos para S100, Melan-A y HMB45, con lo que se estableció el diagnóstico de neurofibroma pigmentado. Conclusiones: Aunque el NP es un subtipo raro del neurofibroma, se considera que es un tumor benigno de evolución crónica de células productoras de melanina. Estas lesiones aparecen en solitario o asociadas con neurofibromatosis. Dado que es un tumor que puede confundirse con otras lesiones cutáneas, es fundamental el análisis de la biopsia para diferenciarlo de otros tumores cutáneos pigmentados, como el schwanoma melanocítico, dermatofibrosarcoma protuberans, hamartoma neurocrístico o neuronevus. La vigilancia es parte del tratamiento y, en ocasiones, se lleva a cabo la resección quirúrgica.
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Pigmented actinic keratosis (PAK) is an uncommon variant of actinic keratosis that can mimic different pigmented lesions, which may be benign or malignant. The diagnosis of PAK is often challenging because of overlapping features with lentigo maligna melanoma (LMM). Clinically, lesions of both conditions almost look similar; the diagnoses must be established histologically and with the help of immunostaining whenever needed. The distinction between a large PAK and LMM is important because their prognosis and management differ. We present a 75-year-old female with annular brown-to-black-colored maculo-plaque on forehead having clinical suspicious of melanocytic malignancy; which was diagnosed with a PAK on biopsy with help of histopathology and confirmed with Melan A/MART‑1 immunostaining.
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The morphological characteristics and biological behaviors of melanocytic nevi in children are different from those in adults. Up to now, the diagnosis and classification of melanocytic nevi in children are still based on international standards, there have been few Chinese studies on their comorbidities, nursing care and psychological effects, and their treatment is also confusing. Based on the relevant literature in China and other countries, and combined with clinical experience, the authors propose the diagnostic process, treatment and nursing suggestions for melanocytic nevi in children, and expect to carry out cooperative research with peers to standardize the diagnosis and treatment of melanocytic nevi in children.
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A 9-year-old girl presented with a symptomless irregular dark brown papule on the vulva for 2 weeks. The patient was initially clinically diagnosed with pigmented nevus, and underwent surgical excision. Postoperative histopathological examination showed dilated glands and tubules in the dermis, eosinophilic secretions and pigment granules in the tubules, decapitation secretion in the inner layer cells, and flat myoepithelial cells around the tubules; a large amount of brown-black pigments were seen in the glands, tubular epithelium and interstitium, and lymphocytes and plasma cells infiltrated the interstitium. Finally, the diagnosis of pigmented apocrine hamartoma was made.
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Objective:To analyze clinical and histopathological features of malignant transformation of congenital melanocytic nevi (CMN) .Methods:From January 2010 to September 2020, 98 patients with clinically and pathologically confirmed malignant transformation of CMN were enrolled from Xijing Hospital, Air Force Medical University, and their clinical and histopathological features as well as immunohistochemical staining and genetic testing results were retrospectively analyzed.Results:Among the 98 patients with malignant transformation of CMN, 45 (45.9%) were males and 53 (54.1%) were females. Their ages ranged from 4 months to 86 years, with a median age of 47 years. The lesions were located on the trunk (34 cases, 34.7%), limbs (25 cases, 25.5%), acra (24 cases, 24.5%), head, face and neck (13 cases, 13.3%), and mucosa (2 cases, 2.0%). Among the 98 patients, 95 (96.9%) had a history of obvious changes in lesions (sudden enlargement, newly developed papules, ulceration, itching, or pain), and the interval time from obvious changes in lesions to diagnosis varied from 2 weeks to 5 years; among the 95 cases, the average age at the onset of obvious changes in lesions was 46 years, and the changes of lesions occurred before the age of 18 years in 4 cases (4.1%), occurred between the age of 18 and 40 years in 35 (35.7%), and occurred after the age of 40 years in 56 (57.1%). In addition, 55 (57.9%) patients experienced a sudden enlargement of primary lesions, 52 (54.7%) developed ulcers in the primary lesions, 21 (22.1%) developed red or black papules or nodules on the surface of primary lesions, 4 (4.2%) developed subcutaneous masses, 2 (2.1%) had itching, and 1 (1.1%) only had pain. The remaining 3 (3.1%) patients experienced slow enlargement of primary lesions. Among the 98 cases of melanoma originating from CMN, 85 (86.7%) arose from small CMN, 11 (11.2%) from medium CMN, and 2 (2.0%) from large CMN. Histopathological examination showed no residual nevus cells in 86 (87.8%) cases, which only had characteristics of typical melanoma; residual nevus cells were only seen in 12 (12.2%) cases, and melanoma cells in the 12 cases all expressed HMB45, while residual nevus cells did not express HMB45 in 11 of 12 cases. Immunohistochemical staining for 5-hydroxymethylcytosine (5hmC) was conducted in 7 cases, and 6 showed negative staining in tumor cells and positive staining in residual nevus cells. BRAF gene detection was conducted in lesional tissue specimens from 22 patients, and it was negative in 1 case of melanoma originating from large CMN and 10 (47.6%) cases of melanoma from small CMN, and positive in 11 (52.4%) cases of melanoma from small CMN.Conclusion:The malignant transformation of CMN mostly occurred on the trunk, and was commonly observed in patients aged over 40 years; most patients had a history of obvious changes (sudden enlargement, newly developed papules, ulceration, etc.) in lesions before diagnosis, and a few patients only felt itching or pain in lesions; immunohistochemical staining of HMB45 and 5hmC could help to distinguish melanoma cells from dermal nevus cells; confirmation of the diagnosis of malignant transformation in CMN should be closely combined with clinical and histopathological results.
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@#Higher dietary intake of polyphenols is associated with a reduced risk of cardiovascular disease (CVD) events and mortality. However, these phytochemicals are predominantly present in fruits and vegetables, which are inadequately consumed by some populations, including Filipinos. This narrative mini-review explores the potential role of polyphenol-containing pigmented rice consumption in modulating risk through a range of mechanisms identified in animal studies. Further, human studies have demonstrated promising but inconsistent effects on risk factors associated with the development of CVD, such as dyslipidemia, hyperglycemia, overweight and obesity. Therefore, this review identifies the need for more clinical trials to examine the effect of pigmented rice on CVD risk factors.
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Cholesterol , Glucose , Polyphenolsالملخص
The temporomandibular joint (TMJ) is frequently imaged in head and neck computed tomography (CT) and magnetic resonance imaging (MRI) studies. Depending on the indication for the study, an abnormality of the TMJ may be an incidental finding. These findings encompass both intra- and extra-articular disorders. They may also be related to local, regional or systemic conditions. Familiarity with these findings along with pertinent clinical information helps narrow the list of differential diagnoses. While definitive diagnosis may not be immediately apparent, a systematic approach contributes to improved discussions between clinicians and radiologists and better patient management.
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Humans , Temporomandibular Joint Disorders/pathology , Incidental Findings , Temporomandibular Joint/pathology , Tomography, X-Ray Computed , Magnetic Resonance Imagingالملخص
Purpose: Palpebral congenital melanocytic nevi (PCMN) is a rare congenital skin lesion affecting the eyelids that can lead to cosmetic and psychological concerns and potential health risks such as malignancy. Several authors have analyzed therapeutical strategies to treat PCMN. However, there was no consensus in the literature. This systematic review aimed to evaluate the effectiveness, safety, and success of treatments of PCMN. Methods: We conducted a systematic review following PRISMA guidelines from October 2022 to April 2023. We included all types of study designs that described or compared PCMN treatments and interventions, as well as histology, recurrence, adverse events, patient satisfaction, and malignant transformation. The search strategy was based on specific search words through the following databases: PubMed, Embase, Latin American and Caribbean Health Sciences Literature (Lilacs), Web of Science, and Scopus. Ongoing studies and gray literature studies were included. Results: We analyzed 25 case reports with 148 participants. The effectiveness, success, and satisfaction with various treatments for PCMN depend on the specific treatment method and the individual patient's case. Conclusions: Most of the studies showed that surgical procedures (exeresis) are able to treat PCMN in the eyelid. The variability in outcomes emphasizes the importance of further research to better understand the most effective and safe approaches for treating congenital melanocytic nevi.
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Skin Abnormalities , Eyelid Neoplasms/therapy , Nevus, Pigmented/therapyالملخص
Resumen ANTECEDENTES: La enfermedad de Paget pigmentada de la mama es una variante poco frecuente de este padecimiento cutáneo, una dermatosis que afecta al complejo areola-pezón de manera típicamente unilateral. La importancia del reconocimiento de esta dermatosis pigmentada inespecífica es que forma parte del diagnóstico diferencial del melanoma cutáneo, por manifestarse como una mácula pigmentada irregular, cuyo estudio histopatológico muestra una proliferación de melanocitos en los estratos suprabasales de la epidermis, fagocitosis de melanina y melanófagos en dermis, hallazgos muy similares al melanoma cutáneo, además de la coexistencia de células claras malignas, características de la enfermedad de Paget. La inmunohistoquímica es una técnica auxiliar en la dermatopatología, que forma parte del proceso diagnóstico de los pacientes para lograr el diagnóstico certero que, al correlacionar la clínica y el estudio histopatológico, le permite al dermatólogo tratar a los pacientes con enfermedad de Paget pigmentada de la mama junto con un equipo multidisciplinario para la búsqueda, diagnóstico y, en su caso, tratamiento quirúrgico de las neoplasias subyacentes que suelen relacionarse con esta variante pigmentada. CASO CLÍNICO: Paciente de 35 años con una lesión pigmentada que afectaba el complejo areola-pezón derecho. Con la correlación histopatológica e inmunohistoquímica se estableció el diagnóstico de enfermedad de Paget pigmentada de la mama y posterior al procedimiento ginecológico, se asoció esta dermatosis con un adenocarcinoma microinvasor triple negativo. CONCLUSIONES: La enfermedad de Paget pigmentada de la mama es un diagnóstico complejo que requiere correlación clínico-patológica y estudios de extensión para valorar su asociación con neoplasias subyacentes.
Abstract BACKGROUND: Pigmented Mammary Paget's Disease is a rare variant of this skin condition, an unilateral dermatosis that typically affects the areola-nipple complex. The importance of recognizing this nonspecific pigmented dermatosis resides in its differential diagnosis of cutaneous melanoma, as it presents as an irregular pigmented macula. The histopathological study shows proliferation of melanocytes in the suprabasal layers of epidermis, phagocytosis of melanin and melanophages in dermis. These findings are very similar to cutaneous melanoma, in addition to the presence of malignant clear epitelial cells that are characteristic of Paget's disease. Immunohistochemistry is part of the approach of patients with lesions that shows proliferating melanocytes to rule out other neoplasms. Making an accurate diagnosis by correlating the clinical, histopathological study and immunohistochemistry allows the dermatologist to approach patients with mammary pigmented mammary Paget's disease with a multidisciplinary team for the diagnosis and surgical treatment of the underlying neoplasms that are usually related to this pigmented variant. CASE REPORT: We report the case of a 35 years old woman with a pigmented lesion that affected the right areola-nipple complex. With the histopathological and immunohistochemical correlation, the diagnosis of pigmented mammary Paget's disease was made and after the gynecological approach, this dermatosis was associated with a triple negative microinvasive adenocarcinoma. CONCLUSION: Pigmented mammary Paget's disease is a complicated diagnosis that requires clinicopathological correlation and extension studies to assess its association to underlying neoplasms.
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Objetivo: Avaliar os casos nos quais os pacientes apresentassem SVNP na ATM, incluindo aspectos clínicos, imaginológicos, histopatológicos e tratamento. Metodologia: Trata-se de uma revisão integrativa com dados obtidos nas bases de dados SciELO, PubMed, Medline e Lillacs entre 1982 e 2021, através dos descritores: "Case report", "Temporomandibular Joint", "Pigmented Villonodular Synovitis". Critérios de inclusão: estudos de relato de caso, textos completos disponíveis, idiomas de publicação em inglês, português ou espanhol. Critérios de exclusão: estudos sem presença de aspectos clínicos, relatos não localizados na ATM, artigos de metanálise, revisão sistemática e de literatura, ensaios clínicos, capítulos de livro, dissertações e teses. Dos 156 resultados, apenas 23 compuseram a revisão. Resultados: Como tratamento, a ressecção total através da cirurgia aberta é recomendada. Os sintomas mais comuns foram: dor na mastigação, trismo, dor pré auricular, dormência, parestesia, perca auditiva e inchaço da glândula parótida. Conclusão: Os aspectos imaginológicos revelam erosão de fossa glenóide e côndilo, histopatologicamente, células gigantes com depósito de hemossiderina, e o tratamento recomendado, ressecção via cirurgia aberta com posterior curetagem... (AU)
Objective: To evaluate the cases in which patients presented PVNS in the TMJ, including clinical, imaging, histopathological and treatment aspects. Methodology: This is an integrative review with data obtained from the SciELO, PubMed, Medline and Lillacs databases between 1982 and 2021, using the descriptors: "Case report", "Temporomandibular Joint", "Pigmented Villonodular Synovitis". Inclusion criteria: case report studies, full texts available, languages of publication in English, Portuguese or Spanish. Exclusion criteria: studies without the presence of clinical aspects, reports not located in the TMJ, meta analysis articles, systematic and literature reviews, clinical trials, book chapters, dissertations and theses. Of the 156 results, only 23 made up the review. Results: As a treatment, total resection through open surgery is recommended. The most common symptoms were: chewing pain, trismus, pre-auricular pain, numbness, paresthesia, hearing loss and parotid gland swelling. Conclusion: The imaging findings reveal erosion of the glenoid fossa and condyle, histopathologically, giant cells with hemosiderin deposits, and the recommended treatment, resection via open surgery with subsequent curettage... (AU)
Objetivo: Evaluar los casos en que los pacientes presentaron SVNP en la ATM, incluyendo aspectos clínicos, imagenológicos, histopatológicos y tratamiento. Metodología: Se trata de una revisión integradora con datos obtenidos de las bases de datos SciELO, PubMed, Medline y Lillacs entre 1982 y 2021, utilizando los descriptores: "Caso clínico", "Articulación temporomandibular", "Sinovitis villonodular pigmentada". Criterios de inclusión: estudios de casos clínicos, textos completos disponibles, idiomas de publicación en inglés, portugués o español. Criterios de exclusión: estudios sin aspectos clínicos, informes no localizados en la ATM, artículos de metanálisis, revisiones sistemáticas y de literatura, ensayos clínicos, capítulos de libros, disertaciones y tesis. De los 156 resultados, 23 conformaron la revisión. Resultados: Como tratamiento se recomienda la resección total mediante cirugía abierta. Los síntomas más frecuentes fueron: dolor masticatorio, trismus, dolor preauricular, entumecimiento, parestesia, hipoacusia e inflamación de glándula parótida. Conclusión: Los hallazgos imagenológicos revelan erosión de fosa glenoidea y cóndilo, histopatológicamente células gigantes con depósitos de hemosiderina y el tratamiento recomendado, resección abierta con posterior curetaje... (AU)
الموضوعات
Humans , Male , Female , Synovitis, Pigmented Villonodular/surgery , Temporomandibular Joint , Giant Cells , Synovitis, Pigmented Villonodular , Curettage , Glenoid Cavityالملخص
RESUMEN Introducción: la melanosis neurocutánea es un trastorno congénito no hereditario que se caracteriza por la asociación de nevus pigmentados múltiples o de gran tamaño y una excesiva proliferación de melanocitos en el sistema nervioso central. La incidencia es similar en ambos sexos, y se observa historia familiar de melanoma en un único caso. Presentación del caso: se trata de un neonato masculino que nace en Hospital General de Luanda en Angola, con mancha melánica gigante que se extiende desde el cuello, cara, tórax, abdomen, espalda y miembros superiores, requiere una vigilancia de las lesiones dérmicas y un control de las crisis convulsivas. Discusión: se realizaron revisiones de la literatura médica disponible sobre el tema, consultando el programa de genética Oxford, y se tomaron fotos de las características clínicas sobresalientes. Por lo general los síntomas neurológicos son de temprana aparición en la etapa neonatal o de lactante con presencia de convulsiones de difícil control, al crear un pronóstico reservado. Conclusiones: se considera importante el seguimiento del neurodesarrollo de forma multidisciplinario para intervención oportuna si fuera necesario.
ABSTRACT Introduction: neurocutaneous melanosis is a non-hereditary congenital disorder characterized by the association of multiple or large pigmented nevi and an excessive proliferation of melanocytes in the central nervous system. The incidence is similar in both sexes, and a family history of melanoma is observed in a single case. Case presentation: this is a male neonate born at the General Hospital of Luanda in Angola, with a giant melanic spot that extends from the neck, face, chest, abdomen, back and upper limbs, requires surveillance of dermal lesions and control of seizures. Discussion: reviews of the available medical literature on the subject were conducted, consulting the Oxford genetics program, and photos of outstanding clinical features were taken. Usually the neurological symptoms are of early onset in the neonatal or infant stage with the presence of seizures that are difficult to control, creating a reserved prognosis. Conclusions: it is considered important to monitor neurodevelopment in a multidisciplinary way for timely intervention if necessary.
RESUMO Introdução: a melanose neurocutânea é uma doença congênita não hereditária caracterizada pela associação de nevi pigmentado múltiplo ou grande e uma proliferação excessiva de melanócitos no sistema nervoso central. A incidência é semelhante em ambos os sexos, e um histórico familiar de melanoma é observado em um único caso. Apresentação do caso: trata-se de um recém-nascido no Hospital Geral de Luanda, em Angola, com um ponto melanico gigante que se estende do pescoço, rosto, tórax, abdômen, costas e membros superiores, requer vigilância de lesões dérmicas e controle de convulsões. Discussão: foram realizadas revisões da literatura médica disponível sobre o tema, consultando o programa de genética de Oxford e fotos de características clínicas de destaque. Geralmente os sintomas neurológicos são de início precoce no estágio neonatal ou infantil com a presença de convulsões de difícil controle, criando um prognóstico reservado. Conclusões: é considerado importante monitorar o neurodesenvolvimento de forma multidisciplinar para intervenção oportuna, se necessário.
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ABSTRACT: Oral mucosal melanoma is an unusual and aggressive malignant tumor that mainly affects the palate of men aged between 50 and 60 years. We present a literature review focusing on the etiopathogenesis and the clinicopathologic features of this entity. In addition, we reported a rare case of an oral mucosal melanoma arising in the left cheek of a 60-yea r- old man. Computed tomography scan revealed infiltration of the tumor to other anatomic structures including the maxillary sinus, the zygomatic bone and the pterygoid processes. Based on its extension, the lesion was considered inoperable and treatment with three-dimensional conformal radiation therapy was proposed but the patient only attended to the first session and died from cancer progression 6 months after the diagnosis. This paper reinforces the importance of inclusion of this malignant tumor in the differential diagnosis of pigmented lesions of the oral mucosa.
RESUMEN: El melanoma de la mucosa oral es un tumor maligno inusual y agresivo que afecta principalmente al paladar de hombres de entre 50 y 60 años. Presentamos una revisión bibliográfica centrada en la etiopatogenia y las características clínico-patológicas de esta entidad. Además, reportamos un caso raro de melanoma de la mucosa oral que surgió en la mejilla izquierda de un hombre de 60 años. La tomografía computarizada reveló la infiltración del tumor a otras estructuras anatómicas, incluido el seno maxilar, el hueso cigomático y los procesos pterigoideos. En base a su extensión, la lesión se consideró inoperable y se propuso tratamiento con radioterapia conformada tridimensional pero el paciente solo asistió a la primera sesión y falleció por progresión del cáncer 6 meses después del diagnóstico. Este trabajo refuerza la importancia de la inclusión de este tumor maligno en el diagnóstico diferencial de las lesiones pigmentadas de la mucosa oral.
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SUMMARY Objective: The aim of this study was to evaluate the expression of endoglin and its correlation with histopathological and clinical findings in conjunctival nevi. Methods: The study included archival formalin-fixed, paraffin-embedded tissue sections of 44 patients with conjunctival nevi. Immunohistochemical staining for CD105 had been performed with monoclonal mouse antihuman CD105 antibodies. The intratumoral microvessel density for quantification of tumoral vascularization had been determined by this marker. Results: The expression of CD105 was positive in 30 (68.2%) cases. There was a statistically significant difference in the level of CD105 expression regarding the histological type of nevus (p=0.03) and intralesional cysts status (p=0.02). Spearman's rho (ρ −0.316) revealed a significant negative correlation between the expression of endoglin and the histological type of nevus (p=0.03) and between the expression of endoglin and the presence of intralesional cysts (ρ −0.380, p=0.01). Conclusion: This study suggests that endoglin could be a useful diagnostic and prognostic marker in differentiating between benign and malignant melanocytic ocular lesions.
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Pigmented transverse nasal band/groove is an asymptomatic benign condition, characterized by the development of erythematous to hyperpigmented, well-demarcated, transverse groove at the junction of middle and lower two-third of the nasal dorsum. Although the pathogenesis is unclear, embryologic origin seems to be the most plausible hypothesis. This condition is often associated other related dermatological conditions such as milia, comedones, seborrheic dermatitis and atopic dermatitis. Diagnosis is mostly clinical, while reassurance is the mainstay of therapy. In persistent cases, topical retinoids have been used. In this article, we have reviewed the different aspects of this condition including treatment, along with the recent updates to create awareness about this dermatological entity