Nevus lipomatosus cutaneous superficialis

Abstract We report a case of nevus lipomatosus cutaneous superficialis of Hoffman-Zurhelle (NCLS), with multiple lesions, in a ten-year-old child. The NLCS is considered rare. The classical clinical presentation is characterized by multiple skin-colored or yellowish papules and nodules, which can have a linear distribution. Histologically, it is characterized by the presence of mature ectopic adipocytes in the dermis. The main therapeutic option is surgical excision. The classical Nevus lipomatosus cutaneous superficialis is reported in this case.

Dermatofibroma simulating seborrheic keratosis dermoscopically.

Dermatofibroma is a frequent benign tumor of easy clinical diagnosis in most cases, but that can mimic other dermatoses. Dermoscopy may help to define the diagnosis and its classical pattern is a central white area, similar to a scar, surrounded by a discrete pigment network. However, dermoscopic findings are not always typical. We describe here a case of dermatofibroma exhibiting ridges, furrows and pseudocomedos, a pattern which is typical of seborrheic keratosis, in dermoscopy.

Dermatofibroma simulating seborrheic keratosis dermoscopically

Abstract: Dermatofibroma is a frequent benign tumor of easy clinical diagnosis in most cases, but that can mimic other dermatoses. Dermoscopy may help to define the diagnosis and its classical pattern is a central white area, similar to a scar, surrounded by a discrete pigment network. However, dermoscopic findings are not always typical. We describe here a case of dermatofibroma exhibiting ridges, furrows and pseudocomedos, a pattern which is typical of seborrheic keratosis, in dermoscopy.

Nevus lipomatosus cutaneous superficialis.

We report a case of nevus lipomatosus cutaneous superficialis of Hoffman-Zurhelle (NCLS), with multiple lesions, in a ten-year-old child. The NLCS is considered rare. The classical clinical presentation is characterized by multiple skin-colored or yellowish papules and nodules, which can have a linear distribution. Histologically, it is characterized by the presence of mature ectopic adipocytes in the dermis. The main therapeutic option is surgical excision. The classical Nevus lipomatosus cutaneous superficialis is reported in this case.

Linear lichen planus in children--case report.

Lichen planus is an uncommon disease in children, and only 2 to 3% of affected patients are under twenty years of age. This dermatosis may appear in several clinical forms, which vary according to the morphology and distribution of lesions. In less than 0.2% of all lichen planus cases, the lesions are distributed along the lines of Blaschko, and is a variant called linear lichen planus. This is a case report of a patient aged two years and eight months, who presented keratotic violaceous papules, affecting the abdomen, buttocks and right thigh, distributed along the lines of Blaschko. Histopathological examination confirmed a diagnosis of linear lichen planus.

Linear lichen planus in children - case report / Liquen plano linear na infancia - relato de caso

Lichen planus is an uncommon disease in children, and only 2 to 3% of affected patients are under twenty years of age. This dermatosis may appear in several clinical forms, which vary according to the morphology and distribution of lesions. In less than 0.2% of all lichen planus cases, the lesions are distributed along the lines of Blaschko, and is a variant called linear lichen planus. This is a case report of a patient aged two years and eight months, who presented keratotic violaceous papules, affecting the abdomen, buttocks and right thigh, distributed along the lines of Blaschko. Histopathological examination confirmed a diagnosis of linear lichen planus.

O líquen plano é uma enfermidade pouco frequente na infância, apenas 2 a 3% dos pacientes acometidos se encontram abaixo dos vinte anos de idade. Essa dermatose pode se apresentar sob várias formas clínicas, as quais variam de acordo com a morfologia e distribuição das lesões. Em menos de 0,2% dos casos de líquen plano, as lesões se distribuem ao longo das linhas de Blaschko, variante denominada de líquen plano linear. Relata-se o caso de uma paciente de dois anos e oito meses de idade apresentando pápulas violáceas de superfície ceratósica acomentendo a região abdominal, região glútea e coxa direita, distribuídas ao longo das linhas de Blaschko, cujo exame histopatológico confirmou o diagnóstico de líquen plano linear.

Pigmented eccrine poroma: report of an atypical case with the use of dermoscopy.

Poromas are uncommon benign neoplasms originating from the terminal ductal portion of the sweat glands, mainly characterized by skin-colored or pink papules or nodules, usually on the extremities. Due to their rarity, the pigmented form clinical hypothesis is hardly ever suggested and psychopathology is fundamental. We report a case of pigmented eccrine poroma in the right palmer area, a location considered atypical. We stress the importance of dermoscopy as a method for diagnosis of poromas, especially in the differential diagnosis with other pigmented nodular-popular lesions.

Pigmented eccrine poroma: report of an atypical case with the use of dermoscopy / Poroma ecrino pigmentado: relato de caso atipico com descricao da dermoscopia

Poromas are uncommon benign neoplasms originating from the terminal ductal portion of the sweat glands, mainly characterized by skin-colored or pink pa pules or nodules, usually on the extremities. Due to their rarity, the pigmented form clinical hypothesis is hardly ever suggested and psychopathology is fundamental. We report a case of pigmented eccrine poroma in the right palmer area, a location considered atypical. We stress the importance of dermoscopy as a method for diagnosis of poromas, especially in the differential diagnosis with other pigmented nodular-popular lesions.

Os poromas são neoplasias benignas incomuns oriundas das porções ductais terminais das glândulas sudoríparas. Caracterizam-se principalmente por pápula ou nódulo cor da pele ou róseo, em geral nas extremidades. Devido à raridade, a hipótese clínica da forma pigmentada dificilmente é aventada, sendo fundamental a histopatologia. Relata-se caso de poroma écrino pigmentado na região palmar direita, localização considerada atípica. Ressalta-se a importância da dermoscopia como método auxiliar no diagnóstico do poroma e, principalmente, no diagnóstico diferencial com outras lesões pápulo-nodulares pigmentadas.

Tratamento dos grânulos de Fordyce com laser de CO2 - Relato de dois casos / Treatment of Fordyce granules with a CO2 laser: report of two cases

Os grânulos de Fordyce são referidos como glândulas sebáceas ectópicas benignas caracterizadas por múltiplas pápulas amareladas, principalmente em lábio superior e observadas na maioria dos casos em homens adultos. Descrevem-se dois casos de pacientes com grânulos de Fordyce que foram submetidos ao tratamento com laser de CO2 obtendo bom resultado cosmético.

Fordyce granules are referred to as benign ectopic sebaceous glands characterized by multiple yellow papules, occurring mainly in the lower lip. It is mainly observed in adult men. The present study describes two cases of patients with Fordyce granules who were treated using a CO2 laser and obtained good cosmetic results.

Cutaneous effects after prolongaded use of hydroxyurea in Polycythemia Vera.

Hydroxyurea is an hydroxylated urea derivative used in many myeloproliferative disorders. Many, but unusual cutaneous disorders are related after its prolonged use. Their pathogenesis is not clear, but it is suggested that there is direct toxicity of the drug on the skin. We described a white, 75-year old man with diagnosis of Polycythemia Vera that in 11 years of treatment developed many cutaneous lesions: skin hyperpigmentation, atrophic lesions on forearms, longitudinal melanonychia of 20 nails, right forearm ulcer, cutaneous xerosis, ichthyosis and auricular spinocellular carcinoma. At this moment, the literature reports describe little diversity of lesions in affected patients.

Efeitos colaterais cutâneos após uso prolongado de hidroxiuréia na Policitemia Vera / Cutaneous effects after prolongaded use of hydroxyurea in Polycythemia Vera

A hidroxiureia é um derivado hidroxilado da ureia utilizado em diversas desordens hematológicas. Inúmeras alterações cutâneas, porém raras, são relatadas após seu uso prolongado. A patogênese das mesmas não está bem esclarecida, porém, sugere-se que a droga tenha uma ação tóxica direta sobre a pele. Descrevemos um homem de 75 anos, branco, com diagnóstico de Policitemia Vera que, ao longo de 11 anos de tratamento com hidroxiureia, evoluiu com várias lesões cutâneas: hiperpigmentação da pele, lesões atróficas em antebraços, melanoníquia longitudinal das 20 unhas, úlcera em antebraço direito, xerose cutânea, ictiose em pernas e carcinoma espinocelular no pavilhão auricular direito. Até o momento, os relatos na literatura descrevem pouca diversidade de lesões nos pacientes acometidos.

Hydroxyurea is an hydroxylated urea derivative used in many myeloproliferative disorders. Many, but unusual cutaneous disorders are related after its prolonged use. Their pathogenesis is not clear, but it is suggested that there is direct toxicity of the drug on the skin. We described a white, 75-year old man with diagnosis of Polycythemia Vera that in 11 years of treatment developed many cutaneous lesions: skin hyperpigmentation, atrophic lesions on forearms, longitudinal melanonychia of 20 nails, right forearm ulcer, cutaneous xerosis, ichthyosis and auricular spinocellular carcinoma. At this moment, the literature reports describe little diversity of lesions in affected patients.

[NRAMP1 gene polymorphisms in individuals with leprosy reactions attended at two reference centers in Recife, northeastern Brazil]. / Polimorfismos do gene NRAMP1 em indivíduos com reações hansênicas, atendidos em dois Centros de Referência no Recife, nordeste do Brasil.

INTRODUCTION: To investigate susceptibility to leprosy reactions, three polymorphisms of the natural resistance-associated macrophage protein (NRAMP1) gene were determined in 201 individuals who were attended at two reference centers in Recife, between 2007 and 2008. Of these, 100 were paucibacillary and 101 were multibacillary. METHODS: The 274C/T, D543N and 1729+55del4 polymorphisms of the NRAMP1 gene were determined using the technique of restriction fragment polymorphism on DNA extracted from peripheral blood. Allelic and genotypic frequencies were estimated by direct counting. RESULTS: The predominant genotypes were: CC (51.8%) for 274C/T; GG (86.6%) for D543N; and +-TGTG (59.9%) for 1729+55del4. The mutant genotype 274 TT predominated in negativity of the reverse reaction (p = 0.03) and in positivity of erythema nodosum leprosum (p = 0.04). CONCLUSIONS: Our results suggest that 274 C/T polymorphism of the NRAMP1 gene may aid in determining the susceptibility to type II reactions among leprosy patients.

[Characteristics of leprosy reactions in paucibacillary and multibacillary individuals attended at two reference centers in Recife, Pernambuco]. / Características epidemiológicas e clínicas das reações hansênicas em indivíduos paucibacilares e multibacilares, atendidos em dois centros de referência para hanseníase, na Cidade de Recife, Estado de Pernambuco.

INTRODUCTION: Significant reactions frequently occur among leprosy cases, and thus a significant proportion of leprosy patients present disabilities and undergo leprosy retreatment. Clinical-epidemiological characterization of reaction patterns is essential for managing such patients. Objective to describe the epidemiological and clinical characteristics of leprosy reactions among paucibacillary and multibacillary individuals. METHODS: In this cross-sectional study, 201 patients with histories of reactions who were attended at two reference centers for leprosy treatment were evaluated. Variables such as initial bacilloscopy, sex, age, skin phototype, origin, clinical presentation, type of treatment, type of reaction, final bacilloscopy index and time of reaction onset in relation to the treatment were evaluated. Statistical analysis was performed using simple frequencies. To calculate risk factors for multibacillary forms, univariate and multivariate analyses were performed. RESULTS: Male sex, age between 30 and 44 years, phototype V, borderline clinical form, regular treatment, type I reaction, neuritis, presence of 10 to 20 nodules and onset of the leprosy reaction during the treatment were the most frequent findings. CONCLUSIONS: Male patients predominated and were associated with greater risk of developing the multibacillary forms. Leprosy reactions occurred most frequently during the treatment. Multibacillary patients were more likely to need leprosy retreatment, and those with type I and type II reactions presented greater frequency of neuritis, lymphadenopathy, arthritis and iritis than did those with isolated reactions.

Polimorfismos do gene NRAMP1 em indivíduos com reações hansênicas, atendidos em dois Centros de Referência no Recife, nordeste do Brasil / NRAMP1 gene polymorphisms in individuals with leprosy reactions attended at two reference centers in Recife, northeastern Brazil

INTRODUÇÃO: Para investigar susceptibilidade às reações hansênicas, três polimorfismos do gene natural resistance-associated macrophage protein (NRAMP1), foram determinados em 201 indivíduos, atendidos em dois centros de referência no Recife, entre 2007 e 2008, sendo 100 paucibacilares e 101 multibacilares. MÉTODOS: A determinação dos polimorfismos 274C/T, D543N e 1729+55del4 do gene NRAMP1 foi realizada utilizando a técnica do polimorfismo de fragmento de restrição em DNA extraído de sangue periférico e as estimativas das freqüências alélicas e genotípicas foram feitas por contagem direta. RESULTADOS: Os genótipos predominantes foram: CC (51,8 por cento) para 274C/T, GG (86,6 por cento) para D543N e +-TGTG (59,9 por cento) para 1729+55del4. O genótipo mutante 274 TT predominou na negatividade da reação reversa (p=0,03) e na positividade do eritema nodoso (p=0,04). CONCLUSÕES: Nossos resultados sugerem que o polimorfismo 274 C/T do gene NRAMP1 pode auxiliar na determinação da susceptibilidade à reação tipo II em indivíduos com hanseníase.

INTRODUCTION: To investigate susceptibility to leprosy reactions, three polymorphisms of the natural resistance-associated macrophage protein (NRAMP1) gene were determined in 201 individuals who were attended at two reference centers in Recife, between 2007 and 2008. Of these, 100 were paucibacillary and 101 were multibacillary. METHODS: The 274C/T, D543N and 1729+55del4 polymorphisms of the NRAMP1 gene were determined using the technique of restriction fragment polymorphism on DNA extracted from peripheral blood. Allelic and genotypic frequencies were estimated by direct counting. RESULTS: The predominant genotypes were: CC (51.8 percent) for 274C/T; GG (86.6 percent) for D543N; and +-TGTG (59.9 percent) for 1729+55del4. The mutant genotype 274 TT predominated in negativity of the reverse reaction (p = 0.03) and in positivity of erythema nodosum leprosum (p = 0.04). CONCLUSIONS: Our results suggest that 274 C/T polymorphism of the NRAMP1 gene may aid in determining the susceptibility to type II reactions among leprosy patients.

Características epidemiológicas e clínicas das reações hansênicas em indivíduos paucibacilares e multibacilares, atendidos em dois centros de referência para hanseníase, na Cidade de Recife, Estado de Pernambuco / Characteristics of leprosy reactions in paucibacillary and multibacillary individuals attended at two reference centers in Recife, Pernambuco

INTRODUÇÃO: As reações são frequentes e importantes no contexto da hanseníase, representando uma significativa parcela de pacientes com incapacidades e submetidos ao retratamento da hanseníase. A caracterização clínico-epidemiológica dos padrões reacionais é primordial para o manejo dos pacientes. O objetivo desse trabalho é descrever as características epidemiológicas e clínicas das reações hansênicas em indivíduos paucibacilares e multibacilares. MÉTODOS: Estudo transversal onde foram avaliados 201 pacientes com história de quadro reacional, atendidos em dois centros de referência para tratamento da hanseníase. Variáveis como baciloscopia inicial, sexo, idade, fototipo, procedência, forma clínica, tipo de tratamento e de reação, índice baciloscópico final e período de surgimento da reação em relação ao tratamento foram avaliados. A análise estatística foi realizada usando-se frequências simples. Para cálculo dos fatores de risco para as formas multibacilares, foram realizadas análises univariada e multivariada. RESULTADOS: Sexo masculino, idade entre 30-44 anos, fototipo V, a forma clínica borderline, tratamento regular, reação tipo I, neurite, presença de 10 a 20 nódulos e surgimento da reação hansênica durante o tratamento foram os achados mais frequentes. CONCLUSÕES: Predominaram os indivíduos do sexo masculino que se associaram a um maior risco de desenvolvimento da forma multibacilar. As reações hansênicas foram mais frequentes durante o tratamento, os pacientes multibacilares foram mais propensos ao retratamento da hanseníase e aqueles com reações tipo I e II, apresentaram maior frequência de neurite, linfadenopatia, artrite e irite do que aqueles com reação isolada.

INTRODUCTION: Significant reactions frequently occur among leprosy cases, and thus a significant proportion of leprosy patients present disabilities and undergo leprosy retreatment. Clinical-epidemiological characterization of reaction patterns is essential for managing such patients. Objective to describe the epidemiological and clinical characteristics of leprosy reactions among paucibacillary and multibacillary individuals. METHODS: In this cross-sectional study, 201 patients with histories of reactions who were attended at two reference centers for leprosy treatment were evaluated. Variables such as initial bacilloscopy, sex, age, skin phototype, origin, clinical presentation, type of treatment, type of reaction, final bacilloscopy index and time of reaction onset in relation to the treatment were evaluated. Statistical analysis was performed using simple frequencies. To calculate risk factors for multibacillary forms, univariate and multivariate analyses were performed. RESULTS: Male sex, age between 30 and 44 years, phototype V, borderline clinical form, regular treatment, type I reaction, neuritis, presence of 10 to 20 nodules and onset of the leprosy reaction during the treatment were the most frequent findings. CONCLUSIONS: Male patients predominated and were associated with greater risk of developing the multibacillary forms. Leprosy reactions occurred most frequently during the treatment. Multibacillary patients were more likely to need leprosy retreatment, and those with type I and type II reactions presented greater frequency of neuritis, lymphadenopathy, arthritis and iritis than did those with isolated reactions.

Mulheres adultas com acne: aspectos comportamentais, perfis hormonal e ultra-sonográfico ovariano / Acne in adult women: behavior, hormone and ovarian ultrasonographic profile

OBJETIVOS: abordar os aspectos comportamentais, o perfil androgênico e as alterações ultra-sonográficas ovarianas de uma amostra de mulheres adultas com acne. MÉTODOS: estudo de série de casos envolvendo uma amostra de 60 mulheres, escolhidas por conveniência. As pacientes foram submetidas a exame clínico, dosagens hormonais e ultra-sonografia para avaliação da morfologia ovariana. Na análise estatística, foi aplicado o Teste de Fischer para análise de correlação entre as variáveis. RESULTADOS: a média de idade foi de 26,5 anos. Todas as pacientes referiram atitudes e sentimentos negativos em relação à acne, sendo os mais freqüentes ansiedade (88,3 por cento) e manipulação constante das lesões (88,3 por cento). O grau de acne mais observado foi o grau II (48,3 por cento). Foram identificados níveis de androgênios fora da faixa de normalidade em 63,3 por cento, com elevação mais freqüente do, dehidroepiandrosterona (57,6 por cento). O padrão ultra-sonográfico mais observado foi o microcístico (74,6 por cento). CONCLUSÕES: as formas clínicas leves de acne predominaram nas mulheres estudadas. Ainda assim, essas apresentaram, com grande freqüência, atitudes e sentimentos negativos em relação à doença. Foram identificados, ainda, modificação dos níveis séricos dos androgênios, bem como do padrão de normalidade do exame ultra-sonográfico.

OBJECTIVES: to assess behavior, androgenic profile and ovarian ultrasound alterations in a sample of adult women with acne. METHODS: case study involving a serie of 60 women selected for convenience. Patients were submitted to clinical exam, hormonal dosages and ultrasound to assess ovarian morphology. Fischer's test for variables correlation analysis was applied. RESULTS: age median was 26.5 years old. All patients attitudes were negative in relation to acne, the more frequent ones were anxiety (88.3 percent) and frequent manipulation of the lesions (88.3 percent). Degree II was the most frequent acne classification determined (48.3 percent). Abnormal androgen levels were identified(63,3 percent), with frequent dehydroepiandrosterone elevation (57 percent.6 percent). The more frequent ultrasound level observed was the monocystic one (74.6 percent). CONCLUSIONS: mild clinical acne was prevalent in the women studied. Nevertheless, they frequently had negative attitudes and feelings related to the disease. Modifications on the circulating androgen levels, as well as on the ovarian ultrasonographic pattern were identifield.

Síndrome sulfônica: relato de um caso / Sulfonic syndrome: a case report

Durante o tratamento da hanseníase, o uso da dapsona e da rifampicina pode levar a transtornos cutâneos e hepáticos significativos. Relata-se o caso de paciente leucodérmica, com 12 anos de idade, portadora de hanseníase indeterminada, que desenvolveu eritrodermia, hepatite e colecistite no curso da segunda semana de tratamento com esquema para paucibacilar (600mg/mês de rifampicina e 100mg/dia de dapsona). O quadro foi diagnosticado como síndrome sulfônica