Sarcoidosis simulating syringomas.

Sarcoidosis is a granulomatous disease of unknown etiology. The skin is commonly affected. Cutaneous manifestations can mimic other diseases and autoimmune disorders. The dermatologist plays a critical role in elucidating the clinical diagnosis and assisting other specialists in the investigation of a systemic disease. We report a patient with typical cutaneous manifestation of sarcoidosis with pulmonary involvement.

Sarcoidosis simulating syringomas / Sarcoidose simulando siringomas

Sarcoidosis is a granulomatous disease of unknown etiology. The skin is commonly affected. Cutaneous manifestations can mimic other diseases and autoimmune disorders. The dermatologist plays a critical role in elucidating the clinical diagnosis and assisting other specialists in the investigation of a systemic disease. We report a patient with typical cutaneous manifestation of sarcoidosis with pulmonary involvement.

A sarcoidose é uma doença granulomatosa de etiologia desconhecida. A pele é comumente afetada. As manifestações cutâneas podem mimetizar outras afecções e desordens auto-imunes. Assim, o dermatologista assume papel fundamental para elucidar o diagnóstico clínico e auxiliar outros especialistas na investigação de uma doença sistêmica. Relatamos um caso de paciente com manifestação cutânea típica de sarcoidose associada com acometimento pulmonar.

Alterações cutâneas no Kwashiorkor: relato de caso de um homem adulto após cirurgia abdominal / Cutaneous manifestations of kwashiorkor: a case report of an adult man after abdominal surgery

Kwashiorkor é um tipo de desnutrição proteico-energética em que há deficiência dietética de proteína, embora a ingestão de calorias se mantenha adequada. As manifestações cutâneas incluem pele xerótica, com aspecto de esmalte descascado, típica coloração avermelhada a branco-acinzentada dos cabelos, o sinal da bandeira e edema mais evidente, nos membros inferiores e na face, dando aspecto de lua cheia. O presente artigo relata o caso de um paciente adulto, do sexo masculino, previamente submetido à duodenopancreatectomia para tratamento de pancreatite crônica associada ao pseudotumor em cabeça de pâncreas que evoluiu com alterações cutâneas de kwashiorkor após tuberculose pulmonar.

Kwashiorkor is a type of protein-energy malnutrition where diet protein deficit is found, in spite of appropriate caloric intake. Cutaneous manifestations include xerosis, with abnormally dry skin that has a flaking enamel paint aspect, a typical red to gray-white hair color, the "flag sign" and more evident edema in lower limbs and face, giving it a full moon appearance. This article reports a case of a male adult patient who had undergone Whipple surgery for treatment of chronic pancreatitis associated with pseudotumor of the pancreatic head that progressed to cutaneous manifestations of kwashiorkor after pulmonary tuberculosis.

Cutaneous manifestations of kwashiorkor: a case report of an adult man after abdominal surgery.

Kwashiorkor is a type of protein-energy malnutrition where diet protein deficit is found, in spite of appropriate caloric intake. Cutaneous manifestations include xerosis, with abnormally dry skin that has a flaking enamel paint aspect, a typical red to gray-white hair color, the "flag sign" and more evident edema in lower limbs and face, giving it a full moon appearance. This article reports a case of a male adult patient who had undergone Whipple surgery for treatment of chronic pancreatitis associated with pseudotumor of the pancreatic head that progressed to cutaneous manifestations of kwashiorkor after pulmonary tuberculosis.

Você conhece esta síndrome? / Do you know this syndrome?

Relatamos um caso típico, em um paciente masculino de 20 anos, da síndrome de Sjögren-Larsson, que é uma doença neurocutânea, autossômica recessiva e incapacitante, caracterizada por ictiose congênita, plegia espástica e retardo mental. É causada pela deficiência da enzima aldeído graxo desidrogenase. Não tem cura, porém a maioria dos pacientes sobrevive até a idade adulta. O tratamento deve ser multidisciplinar e a terapia dermatológica tem o objetivo de aliviar o prurido persistente e a ictiose.

We report a typical case of Sjogren-Larsson syndrome in a male patient, aged 20. The Sjogren-Larsson syndrome is a neurocutaneous, autosomal recessive and disabling condition, characterized by congenital ichthyosis, spastic paraplegia and mental retardation. It is caused by deficiency of the microsomal enzyme fatty aldehyde dehydrogenase. It has no cure, but most patients survive up to an adult age. Treatment should be multidisciplinary and dermatological therapy aims at relieving the persistent itching and ichthyosis.

Do you know this syndrome? Sjogren-Larsson syndrome.

We report a typical case of Sjogren-Larsson syndrome in a male patient, aged 20. The Sjogren-Larsson syndrome is a neurocutaneous, autosomal recessive and disabling condition, characterized by congenital ichthyosis, spastic paraplegia and mental retardation. It is caused by deficiency of the microsomal enzyme fatty aldehyde dehydrogenase. It has no cure, but most patients survive up to an adult age. Treatment should be multidisciplinary and dermatological therapy aims at relieving the persistent itching and ichthyosis.

Congenital toxoplasmosis: late pregnancy infections detected by neonatal screening and maternal serological testing at delivery.

The first aim of this study was to determine the prevalence of congenital toxoplasmosis in newborn infants treated by the public health system in Porto Alegre, a city in southern Brazil, using neonatal screening for Toxoplasma gondii-specific IgM. The second aim was to investigate whether the cases detected by this approach could have been identified by the prenatal screening for antibodies to T. gondii that was performed in the same population. A fluorometric assay was used to analyse T. gondii-specific IgM in filter paper specimens obtained from newborn infants for routine screening for metabolic diseases. When the specific IgM was positive, serum samples from the infant and the mother were requested for confirmatory serological testing, and the infant underwent clinical examination. Among 10 000 infants screened for T. gondii-specific IgM, seven filter paper samples were positive, and congenital toxoplasmosis was confirmed in six patients. The prevalence of IgM specific for T. gondii was 6/10 000 [95% CI 2/10 000, 13/10 000]. One infected infant had already been identified in the maternity ward before birth, three had been identified by maternal serology at delivery, and two infants with congenital toxoplasmosis were identified solely through neonatal screening. Although four mothers of the patients with congenital toxoplasmosis received prenatal care, and three mothers had one or two serological tests for T. gondii-specific antibodies (one at first trimester, one at first and second trimesters, and the other at second and third trimesters), they were not identified during pregnancy as infected. Neonatal screening identified cases of infection not detected by obtaining only one or two serum samples from pregnant women for T. gondii serology, mainly when infection was acquired and transmitted in late pregnancy. Maternal serology at delivery and neonatal screening were especially useful in the identification of infants with congenital toxoplasmosis when the mother did not receive regular prenatal serological testing or prenatal care.

Alterações ungueais como manifestações de doenças sistêmicas / Nails manifestations of systemic diseases

Os autores revisam as manifestações ungueais de diversas doenças sistêmicas, focando os sinais presentes ao exame físico. Proporcionam pistas para o diagnóstico de diversas patologias através da simples inspeção das unhas durante a consulta médica

Comparação entre as concentrações de IgG anti-toxoplasma gondii em recém-nascidos não-infectados e suas mães, no período pós-parto imediato / Comparison of anti-Toxoplasma gondii IgG concentrations at delivery in uninfected newborns and their mothers

O objetivo deste artigo é avaliar a correlação entre as concentrações sérias de IgG anti-Toxoplasma em recém-nascidos (RN) sem infecção congênita e suas mães, logo após o nascimento. Levantamento retrospectivo da sorologia neonatal e materna de crianças no HSL-PUCRS nos últimos 4 anos e acompanhadas no Ambulatório de Infecções Congênitas, por suspeita de toxoplasmose congênita. Critérios de inclusão: 1) pacientes acompanhados até a negativação da sorologia, sendo excluída a infecção congênita; 2) que ao nascimento apresentavam apenas IgG positiva, não tendo recebido tratamento específico para toxoplasmose; 3) sorologias feitas na mãe e no RN pelo mesmo método, no laboratório do Hospital; 4) coleta do sangue materno entre 24 horas antes e 24 horas após o parto; 5) não mais de 72 horas de intervalo entre a coleta do sangue materno e a do RN. Foram calculados os coeficientes de variação dos testes e os valores foram considerados diferentes quando não havia sobreposição dos limites. Cinqüenta e dois pares RN/ mãe foram selecionados para o estudo. Vinte e seis apresentavam valor de IgG anti-Toxoplasma maior, 19 valor igual e 7 valor menor que o da mãe. A regressão linear calculada para a amostra (Y = 1,001X + 35,699) mostrou superioridade dos níveis neonatais quando comparados aos maternos. O nível de IgG anti-Tosoplasma no recém-nascido correlacionou-se positivamente com o materno e a previsibilidade de Y a partis de X apresentou alto nível de significância estatística (P < 0,000). O coeficiente de correlação de Pearson foi 0,86 (correlação forte) e o coeficiente de determinação foi de 0,74. Concluiu-se que mantendo correlação linear e positiva com os níveis maternos, os níveis de IgG anti-Tosoplasma dos RNs semtoxoplasmose congênita apresentaram-se, freqüentemente, superiores aos de suas respectivas mães.