Nevo de Spitz e nevo de Reed

Spitz and Reed nevi, also called spindle and/or epithelioid cell nevi, are a special group of melanocytic lesions due to their peculiar clinical, dermoscopic and histopathological features. The study of these nevi is of great interest because both their nomenclature and classification are still a matter of discussion. Most importantly, the difficulty to differentiate them from melanoma can lead to inadequate therapies. In this context, dermoscopy, a link between clinical and anatomopathological examinations, appears as a helpful diagnostic tool whose accuracy can reach 93%. Borderline lesions are still a great challenge and object of research, including molecular studies. The present study explores the relevant characteristics of these nevi, with emphasis on dermoscopic findings, aiming at understanding their natural history, as well as discussing treatment and patient follow-up.Os nevos de Spitz e de Reed, ou também chamados nevos de células fusiformes e/ou epitelióides, formam um grupo especial de lesões melanocíticas, por suas características peculiares, tanto clínicas como dermatoscópicas e histopatológicas. Torna-se grande o interesse no estudo desses nevos, pois tanto a nomenclatura como a classificação ainda são motivos de debate e, sobretudo, a dificuldade de diferenciação com o melanoma pode gerar condutas terapêuticas inadequadas. Neste contexto, surge a dermatoscopia, elo entre o exame clínico e o anatomopatológico, como método diagnóstico auxiliar importante cuja acurácia pode chegar a 93%. A grande dificuldade ainda são as lesões borderline, motivo de pesquisas, incluindo estudos moleculares. O presente estudo aborda os aspectos relevantes das características destes nevos, com ênfase nos achados dermatoscópicos, buscando o entendimento da sua história natural, além da discussão quanto ao tratamento e seguimento dos pacientes.ABC School of MedicineUniversidade Federal de São Paulo (UNIFESP) Department of Pathology and DermatologyUNIFESP, Department of Pathology and DermatologySciEL

A case of pigmented Bowen's disease

Pigmented Bowen's disease is a rare subtype of Bowen's disease. Clinically it presents as a slow-growing, well-defined, hyperpigmented plaque, and should be included as a differential diagnosis of other pigmented lesions. The authors describe a challenging case of pigmented Bowen's disease with non-diagnostic dermscopy findings.Fed Univ Sao Paulo Unifesp, Dept Dermatol, Sao Paulo, SP, BrazilDermatology Department of the Universidade Federal de São Paulo (UNIFESP) - São Paulo (SP), BrazilPathology Department of the Universidade Federal de São Paulo (UNIFESP) - São Paulo (SP), BrazilWeb of Scienc

Necrobiotic xanthogranuloma without paraproteinemia

Necrobiotic xanthogranuloma is a chronic granulomatous and xantomathous disease, characterized by indurated, nontender, yellowish and erythematous nodules and plaques especially located on the periorbital region. It is commonly associated with paraproteinemia and an increased risk for hematological and lymphoproliferative malignancies. Its pathogenesis remains unclear. Alkylating agents, such as chlorambucil and melphalan may be used to treat the disease with variable success. We report a case of this rare disease in a patient with a solitary tumor and without paraproteinemia.O xantogranuloma necrobiótico é doença crônica granulomatosa e xantomatosa, caracterizada por pápulas e placas infiltradas, eritematosas e amareladas, preferencialmente localizadas na região periorbital. É comum associar-se com paraproteinemia e risco aumentado para malignidades hematológicas e linfoproliferativas. Sua patogênese permanece desconhecida. Agentes alquilantes, como clorambucil e melfalan, podem ser utilizados no tratamento com sucesso variável. Relata-se um exemplo dessa rara doença em paciente com lesão única e sem paraproteinemia.Universidade Federal de São Paulo (UNIFESP) Departamento de Dermatologia Escola Paulista de MedicinaUNIFESP, Depto. de Dermatologia Escola Paulista de MedicinaSciEL

Polypoid melanoma and superficial spreading melanoma different subtypes in the same lesion

Melanoma is a malignant melanocytic neoplasm with high mortality rate, and steadily and universally increasing incidence rates. Polypoid melanoma is considered an exophytic variant of the nodular subtype. The incidence of polypoid melanoma is extremely variable, most likely because of the different criteria used for its characterization. We presented a rare case of polypoid melanoma and superficial spreading melanoma in the same lesion.Universidade Federal de São Paulo (UNIFESP)UNIFESPSciEL

DRESS: características clinicopatológicas em 10 casos de Hospital Universitário em São Paulo

BACKGROUND: Drug reaction with eosinophilia and systemic symptoms is a severe form of drug-induced reaction with multiorgan involvement. OBJECTIVES: The aim of this study is to evaluate the epidemiological, clinical and pathological features and prognosis of this drug reaction among patients seen at a dermatology service. METHOD: Retrospective review of medical records of ten patients diagnosed with drug reaction with eosinophilia and systemic symptoms at the Federal University of São Paulo, from august 2008 to may 2011. RESULTS: Phenytoin was the leading cause of drug reaction with eosinophilia and systemic symptoms in our cases, followed by allopurinol. Abnormal liver function tests were observed in 7 patients and renal function impairment in 2 patients. In all cases, patients were hospitalized and the culprit drug was withdrawn. The main treatment was systemic corticosteroid. Drug reaction with eosinophilia and systemic symptoms resulted in death in 2 cases. The causes of death were septic shock and hepatic failure. CONCLUSION: Our mortality rate of 20%, supports that drug reaction with eosinophilia and systemic symptoms is a severe form of drug-induced reaction and must be recognized by all dermatologists.FUNDAMENTOS: A reação a droga com eosinofilia e sintomas sistêmicos é uma reação medicamentosa severa com envolvimento de múltiplos órgãos. OBJETIVO: Avaliar as características epidemiológicas, clínicas, histológicas e o prognóstico dessa reação medicamentosa entre os pacientes atendidos pelo serviço da dermatologia. MÉTODOS: Levantamento retrospectivo dos prontuários de dez pacientes diagnosticados com reação a droga com eosinofilia e sintomas sistêmicos pelo serviço de dermatologia da Universidade Federal de São Paulo (UNIFESP), entre agosto de 2008 e maio de 2011. RESULTADOS: A fenitoína foi a principal causa de reação a droga com eosinofilia e sintomas sistêmicos entre os pacientes, seguida pelo alopurinol. A alteração das enzimas hepáticas foi observada em sete pacientes e diminuição da função renal em dois casos. Todos os pacientes estavam hospitalizados e o medicamento implicado foi suspenso. O principal tratamento foi corticóide sistêmico. Dois pacientes faleceram devido à síndrome reação a droga com eosinofilia e sintomas sistêmicos. A causa da morte foi choque séptico e falência hepática. CONCLUSÃO: A mortalidade de 20% entre os pacientes do estudo confirma que essa reação induzida por droga é grave e deve ser reconhecida por todos os dermatologistas.Universidade Federal de São Paulo (UNIFESP) Dermatology DepartmentUniversidade Federal de São Paulo (UNIFESP) Dermatology and Pathology DepartmentUNIFESP, Dermatology DepartmentUNIFESP, Dermatology and Pathology DepartmentSciEL

Mammary and extramammary Paget's disease

Paget's disease, described by Sir James Paget in 1874, is classified as mammary and extramammary. the mammary type is rare and often associated with intraductal cancer (93-100% of cases). It is more prevalent in postmenopausal women and it appears as an eczematoid, erythematous, moist or crusted lesion, with or without fine scaling, infiltration and inversion of the nipple. It must be distinguished from erosive adenomatosis of the nipple, cutaneous extension of breast carcinoma, psoriasis, atopic dermatitis, contact dermatitis, chronic eczema, lactiferous ducts ectasia, Bowen's disease, basal cell carcinoma, melanoma and intraductal papilloma. Diagnosis is histological and prognosis and treatment depend on the type of underlying breast cancer. Extramammary Paget's disease is considered an adenocarcinoma originating from the skin or skin appendages in areas with apocrine glands. the primary location is the vulvar area, followed by the perianal region, scrotum, penis and axillae. It starts as an erythematous plaque of indolent growth, with well-defined edges, fine scaling, excoriations, exulcerations and lichenification. in most cases it is not associated with cancer, although there are publications linking it to tumors of the vulva, vagina, cervix and corpus uteri, bladder, ovary, gallbladder, liver, breast, colon and rectum. Differential diagnoses are candidiasis, psoriasis and chronic lichen simplex. Histopathology confirms the diagnosis. Before treatment begins, associated malignancies should be investigated. Surgical excision and micrographic surgery are the best treatment options, although recurrences are frequent.Univ Fed Piaui, Teresina, PI, BrazilUniversidade Federal de São Paulo, São Paulo, SP, BrazilUNISA, Santo Amaro, SP, BrazilHosp Servidor Publ Municipal São Paulo, São Paulo, SP, BrazilFMABC, Santo Andre, SP, BrazilUniversidade Federal de São Paulo, São Paulo, SP, BrazilWeb of Scienc

Liquen nítido geneneralizado associado à síndrome de Down: relato de caso

Lichen nitidus is a disease of unknown etiology, characterized by flesh-colored, shiny papules of 1-2 mm and generally asymptomatic or with mild pruritus. The most common sites of occurrence are genitalia, upper limbs, trunk and abdomen. The generalized form is rare. This is the fourth reported case of lichen nitidus associated with Down Syndrome.Líquen nítido é uma doença de etiologia desconhecida, caracterizada por pápulas normocrômicas, brilhantes medindo de 1 a 2 milímetros de diâmetro, geralmente assintomáticas ou com leve prurido. A forma mais comum é a localizada nos genitais, membros superiores, tronco e abdome. A forma generalizada é rara. Este é o quarto relato de caso descrito de líquen nítido associado à Síndrome de Down.Federal University of São PauloFederal University of São Paulo Dermatology Department Paulista School of MedicineUNIFESP, Dermatology Department Paulista School of MedicineSciEL

Sentinel lymph node in children with melanoma: case report

Objective: to present a case of a child who was subjected to sentinel lymph node biopsy for cutaneous melanoma. Description: a 12 year-old child with Dysplastic Nevus Syndrome developed melanoma on the lumbar region. The excision biopsy revealed a melanoma with depth of 1.5 mm. The patient was submitted to amplification of the margins 2 cm in all directions and the sentinel node was also excised. The histopathological exam did not show residual disease. Sentinel on exam did not show metastases either under hematoxylin-eosin stain or immunohistochemistry (S-100 and HMB45). Therefore, RT-PCR for tyrosinase mRNA was positive. The patient has been followed for twelve months without evidence of recurrence. Comments: childhood melanoma is rare, corresponding to less than 1% of malignant tumors in children. Data point to a worldwide increase in its incidence. Melanoma occurs in melanocytic lesions in 70% and in the remaining 30% it occurs de novo. Melanoma is very aggressive, so the survival depends on an early diagnosis. Sentinel lymph node biopsy has selected patients to complete lymphadenectomy. Some authors have been using this technique in childhood melanoma.Objetivo: apresentar um caso de melanoma cutâneo na infância, submetido à biópsia de linfonodo sentinela. Descrição: criança de 12 anos de idade, portadora da síndrome do nevo displásico, desenvolveu melanoma em dorso. A biópsia excisional revelou melanoma (Breslow = 1,5 mm), sendo submetida a tratamento cirúrgico da lesão, com ampliação de margem de 2cm e biópsia de linfonodo sentinela. O exame anatomopatológico não mostrou doença residual. O exame do linfonodo sentinela não mostrou metástases ao exame histopatológico por hematoxilina eosina, nem à imunohistoquímica (S100 e HMB45). No entanto, a pesquisa de RNA mensageiro da tirosinase por RT-PCR se mostrou positiva. A paciente não mostrou sinais de metástase ou recidiva local nestes doze meses iniciais de seguimento. Comentários: o melanoma é raro em crianças, corresponde a menos de 1% dos tumores da infância. Inúmeros trabalhos mostram o aumento da incidência mundial do melanoma. Em cerca de 70%, se originam de nevos melanocíticos pré-existentes, e nos 30% restantes, eles surgem de novo. Dada a agressividade do melanoma, a sobrevida depende do diagnóstico precoce. A biópsia do linfonodo sentinela tem contribuído na decisão de se realizar ou não a linfadenectomia completa, e alguns autores já estão utilizando a mesma em crianças.Escola Paulista de Medicina Programa de Pós-graduação em Cirurgia PlásticaUniversidade Federal de São Paulo (UNIFESP) Disciplina de Cirurgia PlásticaUniversidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Departamento de PatologiaUNIFESP, EPM, Programa de Pós-graduação em Cirurgia PlásticaEPM, Disciplina de Cirurgia PlásticaEPM, Escola Paulista de Medicina Depto. de PatologiaSciEL

Is intra-operative gamma probe detection really necessary for inguinal sentinel lymph node biopsy?

CONTEXT: Sentinel node (SN) biopsy has changed the surgical treatment of malignant melanoma. The literature has emphasized the importance of gamma probe detection (GPD) of the SN. OBJECTIVE: Our objective was to evaluate the efficacy of patent blue dye (PBD) and GPD for SN biopsy in different lymphatic basins. DESIGN: Patients with cutaneous malignant melanoma in stages I and II were submitted to biopsy of the SN, identified by PBD and GPD, as part of a research project. SETTING: Patients were seen at Hospital São Paulo by a multidisciplinary group (Plastic Surgery Tumor Branch, Nuclear Medicine and Pathology). PATIENTS: 64 patients with localized malignant melanoma were studied. The median age was 46.5 years. The primary tumor was located in the neck, trunk or extremities. INTERVENTIONS: Preoperative lymphoscintigraphy, lymphatic mapping with PBD and intraoperative GPD was performed on all patients. The SN was examined by conventional and immunohistochemical staining. If the SN was not found or contained micrometastases, only complete lymphadenectomy was performed. MAIN MEASUREMENTS: The SN was identified by PBD if it was blue-stained, and by GPD if demonstrated activity five times greater than the adipose tissue of the neighborhood. RESULTS: Seventy lymphatic basins were explored. Lymphoscintigraphy showed ambiguous drainage in 7 patients. GPD identified the SN in 68 basins (97%) and PBD in 53 (76%). PBD and GPD identified SN in 100% of the inguinal basins. For the remaining basins both techniques were complementary. A metastatic SN was found in 10 basins. Three patients with negative SN had recurrence (median follow-up = 11 months). CONCLUSION: Although both GPD and PBD are useful and complementary, PBD alone identified the SN in 100% of the inguinal lymphatic basins.CONTEXTO: A biópsia de linfonodo sentinela (LS) mudou a abordagem cirúrgica do melanoma maligno. A literatura tem enfatizado a importância da detecção gama intra-operatória (DG) do LS. OBJETIVO: Nosso objetivo é avaliar a eficácia do corante azul patente (AP) e da DG na biópsia de LS em diferentes bases linfáticas. TIPO DE ESTUDO: Pacientes portadores de melanoma maligno cutâneo foram submetidos à biópsia do LS, usando AP e DG como parte de um projeto de pesquisa. LOCAL: Hospital São Paulo, grupo multidisciplinar (cirurgião oncológico, médico nuclear e patologista). PACIENTES: Foram estudados 64 pacientes portadores de melanoma maligno localizado, com idade mediana de 46,5 anos. O sítio primário estava localizado no pescoço, tronco e nos membros. INTERVENÇÕES: Linfocintilografia pré-operatória, mapeamento linfático com AP e DG foram realizados em todos os pacientes. O LS foi examinado por histopatologia convencional e imunohistoquímica. Quando o LS não foi encontrado ou continha micrometástases, linfadenectomia completa da base linfática foi realizada. VARIÁVEIS ESTUDADAS: O LS foi considerado como identificado pelo AP se corado em azul e pela DG quando demonstrou pelo menos 5 vezes mais atividade do que o tecido gorduroso vizinho. RESULTADOS: Foram exploradas 70 bases linfáticas. A linfocintilografia mostrou drenagem ambígua em 7 pacientes. DG identificou o LS em 68 bases linfáticas (97%) e o AP o fez em 53 bases (76%). Os dois métodos identificaram separadamente 100% dos LS inguinais. Nas demais bases, as técnicas foram complementares. O LS estava invadido por células tumorais em 10 bases. Três pacientes com LS negativo apresentaram recorrência (seguimento mediano de 11 meses). CONCLUSÃO: Embora o emprego de AP e GP na pesquisa de LS sejam complementares, o AP demonstrou ser um método suficiente para a localização do LS inguinal.Universidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Discipline of Plastic Surgery/Tumor branchUniversidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Department of DermatologyUniversidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Department of Nuclear MedicineUNIFESP, EPM, Discipline of Plastic Surgery/Tumor branchUNIFESP, EPM, Department of DermatologyUNIFESP, EPM, Department of Nuclear MedicineSciEL