Epidemiological and clinical study of cases of endemic pemphigus foliaceus and pemphigus vulgaris in a reference center in the state of Minas Gerais, Brazil

Abstract Background: Pemphigus constitutes a group of autoimmune bullous diseases. A reduction in the incidence of endemic pemphigus foliaceus and an increase in pemphigus vulgaris has been described, but there are no studies in Minas Gerais that address the subject. Objective: To describe the epidemiological and clinical profile of patients with pemphigus treated at the Dermatology Service of a public University Hospital in the state of Minas Gerais, Brazil. Methods: An observational, descriptive, and cross-sectional study was carried out of cases of endemic pemphigus foliaceus and pemphigus vulgaris, for a period of six months. A questionnaire was filled out with epidemiological and clinical data on the disease. Results: A total of 122 patients were included in the study, 64 with endemic pemphigus foliaceus and 58 with pemphigus vulgaris. When comparing patients with endemic pemphigus foliaceus and those with pemphigus vulgaris, a statistical difference was observed between the median age of initial disease manifestation (p = 0.001), patient occupation (p = 0.010), area of residence (p = 0.000), forests (p = 0.000) and rivers/streams close to the dwelling (p = 0.001) and the number of systemic medications required to control the disease (p = 0.002). When comparing patients with endemic pemphigus foliaceus to those evaluated in a study carried out at the same service in 2008, there was a statistical difference in the area of residence (p = 0.030). Study limitations: The assessed population comes from a tertiary care service that is not a reference for the entire state. Conclusions: Patients with endemic pemphigus foliaceus and pemphigus vulgaris maintain statistically significant differences regarding their main variables in the literature, such as age and area of residence. Historically, there has been a reduction in cases of endemic pemphigus foliaceus and an increase in cases of pemphigus vulgaris in this population.

Extradigital glomus tumor: dermoscopic description and histopathological correlation

Abstract Glomus tumors are rare benign neoplasms arising from the neuromyoarterial structure called glomus body. They present as angiomatous papules, soft and painful, especially to cold and pressure. In general, they are solitary and affect the extremities, located mainly the subungual bed. Extradigital lesions are rare and can constitute a diagnostic challenge. This is the report of a patient with an extradigital lesion on the left arm, and its dermoscopic aspects, including angiomatous lagoons circumscribed by a pale halo, a structure not previously described in the two reports of extradigital glomus tumor with dermoscopic features, found in the literature.

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.

Wound myiasis: the role of entodermoscopy

Abstract: Although wound or traumatic myiasis is common in tropical countries, only recently cases associated with underlying dermatoses, such as seborrheic dermatitis and psoriasis, have been reported. We describe a patient with seborrheic dermatitis and an ulcerated lesion on the scalp, in which the dermatological examination with the aid of dermoscopy allowed the identification of larvae (maggots) compatible with infestation by Cochliomyia hominivorax. Treatment was performed with oral and topical ivermectin, followed by manual extraction of the larvae.

Prognostic factors for metastasis in cutaneous melanoma

Abstract: Background: Melanoma is a malignant neoplasia that shows high mortality when diagnosed in advanced stages. Early identification of high-risk patients for the development of melanoma metastases is the main strategy to reduce mortality. Objective: To assess the influence of eight epidemiological and histopathologic features on the development of metastases in patients diagnosed with primary cutaneous melanoma. Methods: Our historical cohort comprised patients with invasive primary cutaneous melanoma seen between 1995 and 2012 at a public university hospital and a private oncologic surgery institution in Southeastern Brazil. The following variables were analyzed: gender, age, family history of melanoma, site of the primary tumor, clinical and histologic subtype, Breslow thickness, histologic ulceration and the mitotic index. Kaplan-Meier univariate test and multivariate Cox proportional hazard analysis were used to assess factors associated with disease-free survival. Results: Five hundred and fourteen patients were enrolled. The univariate analysis identified the following significant risk factors: gender, age, site of the tumor, clinical and histologic subtype, Breslow thickness, histologic ulceration and mitotic index. Multivariate analysis included 244 patients and detected four significant prognostic factors: male gender, nodular clinical and histologic subtype, Breslow thickness > 4mm, and histologic ulceration. The mitotic index was not included in this analysis. Study limitations: Small number of patients in multivariate analysis. Conclusions: The following prognostic factors to the development of melanoma metastasis were identified in the study: male gender, nodular histologic subtype, Breslow thickness > 4mm and ulceration.

A prospective study of patients with large congenital melanocytic nevi and the risk of melanoma

Abstract: Background: Large congenital melanocytic nevus (LCMN) is considered a risk factor for melanoma, although the magnitude of this risk is controversial. Objective: To evaluate the risk of melanoma development in patients with LCMN seen at a dermatology referral center in Brazil during a twelve-year period. To the best of our knowledge, there are no published similar studies on large congenital melanocytic nevus in South America. Methods: Our prospective cohort included only patients with congenital nevi ≥20cm. The cumulative risk of developing melanoma and the standardized morbidity ratio were calculated for patients followed up prospectively for at least 1 month. Results: Sixty-three patients were enrolled in this study. One patient who developed melanoma prior to enrollment was excluded, and five were eliminated because of insufficient follow-up time. Mean follow-up for the remaining 57 patients was 5.5 years (median 5.2 years). Median age of entry into the study was 2.6 years. Most patients (75.4%) underwent only clinical observation. Melanomas occurred in 2 (3.5%) patients. Five-year cumulative risk for melanoma was 4.8% (95% CI: 1.9-11.5%). Standardized morbidity ratio was 1584 (95% CI: 266-5232, p<0.001). Study limitations: The small sample size reduces the accuracy of risk estimates. Conclusions: This study analyzed prospectively for the first time data from South America demonstrating that patients with LCMN have a higher risk of developing melanoma than the general population (p<0.001).

Brazilian guidelines for diagnosis, treatment and follow-up of primary cutaneous melanoma - Part II

Abstract The last Brazilian guidelines on melanoma were published in 2002. Development in diagnosis and treatment made updating necessary. The coordinators elaborated ten clinical questions, based on PICO system. A Medline search, according to specific MeSH terms for each of the 10 questions was performed and articles selected were classified from A to D according to level of scientific evidence. Based on the results, recommendations were defined and classified according to scientific strength. The present Guidelines were divided in two parts for editorial and publication reasons. In this second part, the following clinical questions were answered: 1) which patients with primary cutaneous melanoma benefit from sentinel lymph node biopsy? 2) Follow-up with body mapping is indicated for which patients? 3) Is preventive excision of acral nevi beneficious to patients? 4) Is preventive excision of giant congenital nevi beneficious to patients? 5) How should stages 0 and I primary cutaneous melanoma patients be followed?.

Guidelines of the Brazilian Dermatology Society for diagnosis, treatment and follow up of primary cutaneous melanoma - Part I

Abstract: The last Brazilian guidelines on melanoma were published in 2002. Development in diagnosis and treatment made updating necessary. The coordinators elaborated ten clinical questions, based on PICO system. A Medline search, according to specific MeSH terms for each of the 10 questions was performed and articles selected were classified from A to D according to level of scientific evidence. Based on the results, recommendations were defined and classified according to scientific strength. The present Guidelines were divided in two parts for editorial and publication reasons. In the first part, the following clinical questions were answered: 1) The use of dermoscopy for diagnosis of primary cutaneous melanoma brings benefits for patients when compared with clinical examination? 2) Does dermoscopy favor diagnosis of nail apparatus melanoma? 3) Is there a prognostic difference when incisional or excisional biopsies are used? 4) Does revision by a pathologist trained in melanoma contribute to diagnosis and treatment of primary cutaneous melanoma? What margins should be used to treat lentigo maligna melanoma and melanoma in situ?.

Comorbidities and cardiovascular risk factors in patients with psoriasis

BACKGROUND: Psoriasis is a chronic inflammatory disease and its pathogenesis involves an interaction between genetic, environmental, and immunological factors. Recent studies have suggested that the chronic inflammatory nature of psoriasis may predispose to an association with other inflammatory diseases, especially cardiovascular diseases and metabolic disorders. OBJECTIVES: To describe the demographic, clinical, epidemiological, and laboratory characteristics of a sample of psoriasis patients; to assess the prevalence of cardiovascular comorbidities in this group of patients; and to identify the cardiovascular risk profile using the Framingham risk score. METHODS: We conducted a cross-sectional study involving the assessment of 190 patients. Participants underwent history and physical examination. They also completed a specific questionnaire about epidemiological data, past medical history, and comorbidities. The cardiovascular risk profile was calculated using the Framingham risk score. RESULTS: Patients' mean age was 51.5 ± 14 years, and the predominant clinical presentation was plaque psoriasis (78.4%). We found an increased prevalence of systemic hypertension, type 2 diabetes, metabolic syndrome, and obesity. Increased waist circumference was also found in addition to a considerable prevalence of depression, smoking, and regular alcohol intake. Patients' cardiovascular risk was high according to the Framingham risk score, and 47.2% of patients had moderate or high risk of fatal and non-fatal coronary events in 10 years. CONCLUSIONS: Patients had high prevalence of cardiovascular comorbidities, and high cardiovascular risk according to the Framingham risk score. Further epidemiological studies are needed in Brazil for validation of our results. .

Giant congenital melanocytic nevus / Nevo melanocitico congenito gigante

Giant congenital melanocytic nevus is usually defined as a melanocytic lesion present at birth that will reach a diameter ≥ 20 cm in adulthood. Its incidence is estimated in <1:20,000 newborns. Despite its rarity, this lesion is important because it may associate with severe complications such as malignant melanoma, affect the central nervous system (neurocutaneous melanosis), and have major psychosocial impact on the patient and his family due to its unsightly appearance. Giant congenital melanocytic nevus generally presents as a brown lesion, with flat or mammilated surface, well-demarcated borders and hypertrichosis. Congenital melanocytic nevus is primarily a clinical diagnosis. However, congenital nevi are histologically distinguished from acquired nevi mainly by their larger size, the spread of the nevus cells to the deep layers of the skin and by their more varied architecture and morphology. Although giant congenital melanocytic nevus is recognized as a risk factor for the development of melanoma, the precise magnitude of this risk is still controversial. The estimated lifetime risk of developing melanoma varies from 5 to 10%. On account of these uncertainties and the size of the lesions, the management of giant congenital melanocytic nevus needs individualization. Treatment may include surgical and non-surgical procedures, psychological intervention and/or clinical follow-up, with special attention to changes in color, texture or on the surface of the lesion. The only absolute indication for surgery in giant congenital melanocytic nevus is the development of a malignant neoplasm on the lesion.

O nevo melanocítico congênito gigante é, geralmente, definido como lesão melanocítica presente ao nascimento e que atinge, no mínimo, 20 cm de diâmetro na vida adulta. Sua incidência é estimada em menos de 1:20.000 recém-nascidos. Contudo, apesar de sua raridade, possui importância tanto por estar associado a complicações graves, como o melanoma maligno e o acometimento do sistema nervoso central (melanose neurocutânea), quanto pelo grande impacto psicossocial que ocasiona no paciente e nos familiares, devido a seu aspecto comumente inestético. O nevo congênito gigante, geralmente, apresenta-se como lesão acastanhada, plana ou elevada, de bordas bem definidas e com hipertricose, e seu diagnóstico é eminentemente clínico. Do ponto de vista histológico, porém, os nevos melanocíticos congênitos são diferenciados dos nevos adquiridos, principalmente pelo seu tamanho maior, pela disseminação das células névicas para as camadas mais profundas da pele e pela sua arquitetura e morfologia mais variadas. O nevo congênito gigante é considerado fator de risco para o desenvolvimento do melanoma. Todavia, a real incidência de malignização ainda é controversa. Estima-se que o risco de melanoma ao longo da vida esteja entre 5 e 10%. Diante dessas incertezas e do tamanho das lesões, a abordagem do nevo gigante representa um desafio e deve ser individualizada. O tratamento pode incluir procedimentos cirúrgicos ou não cirúrgicos, intervenções psicológicas e/ou acompanhamento clínico, com atenção a mudanças de coloração, superfície ou textura do nevo. Considera-se que a única indicação absoluta para a intervenção cirúrgica é o surgimento de uma neoplasia maligna sobre a lesão.

Epidemiologia e análise de sobrevida de pacientes com melanoma metastáticode sítio primário conhecido e desconhecido / Epidemiology and analysis of survival of patients with known and unknown primary melanoma site

Introdução: O melanoma é essencialmente cutâneo. Em alguns pacientes, não é possível determinar a localização do tumor primário. A incidência de melanoma com sítio primário desconhecido varia de 2 a 15%. Objetivos: Determinar se há diferença de sobrevida global entre os pacientes com melanoma primário conhecido comparado aos pacientes com melanoma primário desconhecido. Métodos: Foi realizada análise retrospectiva de pacientes com melanoma no banco de dados da Oncologia Cirúrgica e Cirurgia do Aparelho Digestivo (ONCAD) e identificados aqueles com sítio primário desconhecido e metástases para diferentes locais. Resultados: O principal local de metástases foi o linfonodo (50%) - inguinais (25%),axilares (16%) e periaórticos (8,3%). Metástases pulmonares foram encontradas em três pacientes (25%). Metástases para fígado, osso e pele foram observadas em um caso cada (8,3%). Conclusão: A evolução clínica dos pacientes metastáticos com melanoma de sítio primário desconhecido é melhor em relação aos pacientes metastáticos com lesão primária conhecida, quando os dois grupos estão no mesmo estádio. Dessa forma, o fator mais determinante do curso clínico e do prognóstico éa localização das metástases. A maioria dos pacientes que apresenta doença sistêmica ao diagnóstico perde a chance de cura, como muitos pacientes com cutâneo primário fino e doença regional ao diagnóstico.

Introduction: Melanoma is essentially cutaneous. In some patients, it is not possible to determine the location of the primary tumor. The incidence of melanoma of unknown primary site varies from 2 ? 15%. Objectives: To determine whether there is difference in overall survival between patients with known primary melanoma compared to patients with unknown primary melanoma. Methods: A retrospective analysis of patients with melanoma in the database of Surgical Oncology and Digestive Surgery (ONCAD) was performed and identified those with unknown primary site and metastases to different locations. Results: Lymph node was the main site of metastases (50%) - inguinal (25%), axillary (16%) and periaortic (8.3%). Pulmonary metastases were found in three patients ( 25%). Metastasis to the liver, bone and skin were observed in one case each (8.3%). Conclusions: The clinical course of patients with metastatic melanomaof unknown primary site is better than metastatic patients with known primary lesion, when both groups are on the same stage. Thus, the most relevant determining factor affecting clinical course and prognosis is the metastasis location. Most patients presenting systemic disease at diagnosis loses the chance of cure as many patients with thin primary cutaneous and regional disease at diagnosis.

Epidemiological aspects of melanoma at a university hospital dermatology center over a period of 20 years / Aspectos epidemiológicos do melanoma em serviço de dermatologia de hospital universitário em um período de 20 anos

BACKGROUND: The incidence of melanoma has been steadily rising in past decades. Although it accounts for only 3% of all skin cancers, it is responsible for 75% of deaths. OBJECTIVE: to describe the epidemiological aspects of melanoma in a university hospital setting over a period of 20 years. METHODS: A total of 166 patients were analyzed between January 1990 and January 2010 for clinical and histological variables and correlations between them. A 5% level of significance was adopted. RESULTS: The majority of patients were Caucasians (74%), females (61%), with a mean age at diagnosis of 55. The predominant histological type was lentigo maligna/lentigo maligna melanoma (35.7%) and the head and neck was the most affected site (30.7%). Among non-Caucasians, the acral region was the most affected. Most tumors were in situ (41.1%). Growth of the lesion was the most frequent complaint (58.1%) and bleeding was most frequently associated with melanomas with a depth > 4mm. There were seven deaths (4.2%), with a high risk among men, non-Caucasians and those under 20 years of age, with a Breslow's depth > 2mm, with lentiginous acral melanoma and with a history of growth and bleeding. CONCLUSIONS: Our sample differs from most of the studies in the predominant location (head and neck), histological type (lentigo maligna/ lentigo maligna melanoma) and a major risk of death under the age of 20, which could be with a reflex of regional variation. Broader studies are necessary for validation of the results. .

FUNDAMENTOS: A incidência do melanoma cutâneo aumentou nas últimas décadas. Embora represente 3% dos tumores cutâneos, é responsável por 75% dos óbitos. O diagnóstico precoce constitui a principal chance de cura. OBJETIVO: Descrever os aspectos epidemiológicos do melanoma em hospital universitário em 20 anos. MÉTODOS: Avaliaram-se 166 pacientes no período de janeiro de 1990 a janeiro de 2010, quanto s variáveis epidemiológicas, histológicas e óbitos relacionados ao melanoma e suas correlações. Adotou-se nível de significância de 5%. RESULTADOS: A maioria dos pacientes era brancos (74%), mulheres (61%), com média de idade ao diagnóstico de 55 anos. O tipo histológico predominante foi o lentigo maligno/lentigo maligno melanoma (35,7%) e a localização mais frequente foi a cabeça e o pescoço (30,7%). Entre os não-brancos, a região acral foi a mais acometida. Quanto espessura tumoral, a maioria dos melanomas era in situ (41,1%). O crescimento da lesão foi a queixa mais frequente (58%) e o sangramento foi mais associado a melanomas espessos. Ocorreram sete óbitos (4,2%), com maior risco de morte em menores de 20 anos e naqueles com história de sangramento, após análise multivariada. CONCLUSÃO: Esta casuística difere da maioria dos estudos em relação localização (cabeça e pescoço), ao tipo histológico (lentigo maligno/lentigo maligno melanoma) e ao maior risco de óbito em menores de 20 anos, o que pode ser devido variação regional. Estudos mais amplos são necessários para validação destes resultados. .

Case for diagnosis / Caso para diagnóstico

An 80-year-old Caucasian male patient was referred for evaluation of a rapidly growing, asymptomatic, erythematous nodule measuring 2 cm in diameter on his left cheek. The lesion had been present for four months. Dermoscopy revealed a homogeneous pink background with polymorphous telangiectatic vessels. Histopathology showed tumors in the deep dermis and subcutis composed of round cells with scant cytoplasm. Immunohistochemical staining was positive for CK20 confirming the diagnosis of Merkel cell carcinoma.

Paciente de 80 anos, branco, sexo masculino, encaminhado para avaliação de nódulo eritematoso de 2 cm, assintomático, de crescimento rápido, localizado na região malar esquerda, com quatro meses de evolução. À dermatoscopia visualizou-se fundo homogêneo róseo com telangiectasias polimorfas. O exame anatomopatológico revelou massas tumorais atingindo a derme profunda e o subcutâneo, compostas por células arredondadas, de citoplasma escasso. A imunohistoquímica foi positiva para CK20, confirmando o diagnóstico de carcinoma de células de Merkel.

Síndrome do nevo de Becker: relato de caso / Becker's nevus syndrome: case report

O nevo de Becker se manifesta como uma mácula hipercrômica, localizada predominantemente no tronco anterior ou região escapular, e frequentemente associada à hipertricose. A coexistência do nevo de Becker e anormalidades cutâneas, músculo-esqueléticas ou maxilo-faciais corresponde à síndrome do nevo de Becker. Relata-se o caso de uma paciente de 16 anos, portadora desde os sete anos de mácula hipercrômica, localizada no tronco ântero-lateral direito, estendendo-se até a face medial da coxa direita, acompanhada de hipoplasia mamária ipsilateral. O exame anatomopatológico confirmou a hipótese clínica de nevo de Becker.

Becker's nevus is a hyperpigmented macula that is predominantly located on the anterior trunk or on the scapular region, frequently associated with hypertrichosis. The association of Becker's nevus with other cutaneous, musculoskeletal or maxillofacial anomalies has been called Becker's nevus syndrome. We report a case of a 16-year-old girl with a hyperpigmented macula which spread from her right anterolateral trunk to the inner part of her right thigh accompanied by ipsilateral mammary hypoplasia. The skin lesion started when the patient was seven years old when it was also noticed ipsilateral mammary hypoplasia. The histological exam confirmed the clinical hypothesis of Becker's nevus.

Caso para diagnóstico / Case for diagnosis

O termo cútis tricolor descreve a presença de máculas cutâneas hiper e ipocrômicas, adjacentes e congênitas, e é manifestação de mosaicismo cutâneo explicada pelo mecanismo de twin spotting alélico. Esse fenômeno consiste em forma particular de recombinação somática, com perda da heterozigose, que ocorre durante a mitose no embrião em formação. Apresenta-se caso de paciente masculino, 16 anos, com máculas hiper e hipocrômicas adjacentes no tronco, presentes desde o nascimento, assintomáticas. Não se detectaram outras anomalias.

The term cutis tricolor describes the presence of congenital hyper and hypopigmented skin macules and is a manifestation of skin mosaicism, explained by the allelic twin spotting mechanism. This phenomenon consists of a peculiar type of somatic recombination, with loss of heterozygosity, which occurs during mitosis in the forming embryo. We present the case of a male patient, aged 16, with congenital asymptomatic adjacent hyper and hypopigmented macules on the trunk. No other anomalies could be detected.

Manifestações cutâneas decorrentes do uso de drogas ilícitas / Skin manifestations of illicit drug use

O uso e abuso de drogas ilícitas é um problema significativo e de abrangência mundial. A Organização das Nações Unidas estima que 5% da população mundial entre os 15 e 64 anos fazem uso de drogas pelo menos uma vez por ano (prevalência anual), sendo que metade destes usam regularmente, isto é, pelo menos uma vez por mês. Muitos dos eventos adversos das drogas ilícitas surgem na pele, o que torna fundamental que o dermatologista esteja familiarizado com essas alterações.

Illicit drug use and abuse is a major problem all over the world. The United Nations estimates that 5% of world population (aged 15-64 years) use illicit drugs at least once a year (annual prevalence) and half of them use drugs regularly, that is, at least once a month. Many adverse events of illicit drugs arise on the skin and therefore dermatologists should be aware of these changes.