RESUMEN
OBJECTIVE: To evaluate the influence of environmental factors and prematurity relating to juvenile dermatomyositis (JDM), its course and refractoriness to treatment. METHODS: A case-control study with 35 patients followed up at a tertiary hospital and 124 healthy controls, all residents of São Paulo. Patients were classified according to monocyclic, polycyclic or chronic disease courses and refractoriness to treatment. The daily concentrations of pollutants (inhalable particulate matter-PM10, sulfur dioxide-SO2, nitrogen dioxide-NO2, ozone-O3 and carbon monoxide-CO) were provided by the Environmental Company of São Paulo. Data from the population were obtained through a questionnaire. RESULTS: Fifteen patients had monocyclic courses, and 19 polycyclic/chronic courses. Eighteen patients were refractory to treatment. Maternal occupational exposure to inhalable agents (OR = 17.88; IC 95% 2.15-148.16, p = 0.01) and exposure to O3 in the fifth year of life (third tertile > 86.28µg/m3; OR = 6.53, IC95% 1.60-26.77, p = 0.01) were risk factors for JDM in the multivariate logistic regression model. The presence of a factory/quarry at a distance farther than 200 meters from daycare/school (OR = 0.22; IC 95% 0.06-0.77; p = 0.02) was a protective factor in the same analysis. Prematurity, exposure to air pollutants/cigarette smoke/sources of inhalable pollutants in the mother's places of residence and work during the gestational period were not associated with JDM. Prematurity, maternal exposure to occupational pollutants during pregnancy as well as patient's exposure to ground-level pollutants up to the fifth year of life were not associated with disease course and treatment refractoriness. CONCLUSION: Risk factors for JDM were maternal occupational exposure and exposure to O3 in the fifth year of life.
Asunto(s)
Dermatomiositis , Exposición Profesional , Material Particulado , Humanos , Dermatomiositis/etiología , Femenino , Estudios de Casos y Controles , Masculino , Factores de Riesgo , Material Particulado/análisis , Material Particulado/efectos adversos , Niño , Brasil/epidemiología , Exposición Profesional/efectos adversos , Exposición Profesional/análisis , Embarazo , Ozono/análisis , Ozono/efectos adversos , Exposición Materna/efectos adversos , Monóxido de Carbono/análisis , Dióxido de Nitrógeno/análisis , Dióxido de Nitrógeno/efectos adversos , Dióxido de Azufre/análisis , Dióxido de Azufre/efectos adversos , Preescolar , Contaminantes Atmosféricos/análisis , Contaminantes Atmosféricos/efectos adversos , Exposición a Riesgos Ambientales/efectos adversos , Modelos Logísticos , Nacimiento PrematuroRESUMEN
OBJECTIVE: To deep sequence the TRIM33 gene in tumours from patients with cancer-associated anti-TIF1γ autoantibody-positive dermatomyositis (DM) as TRIM33 somatic mutations in tumours may trigger this auto-immune disease. METHODS: Next generation sequencing of tumour DNA samples from patients with cancer-associated anti-TIF1γ autoantibody-positive DM. Fourteen tumours from 13 anti-TIF1γ autoantibody-positive DM individuals were sequenced along with two control tumours from non-DM individuals. RESULTS: Fourteen probable somatic variants from four tumours were identified in the TRIM33 gene. CONCLUSION: These results are in accordance with the previous report of Pinal-Fernandez et al. and support the hypothesis of a role of TRIM33 gene mutations in the pathophysiology of anti-TIF1γ autoantibody-positive DM.
Asunto(s)
ADN/genética , Dermatomiositis/genética , Secuenciación de Nucleótidos de Alto Rendimiento/métodos , Mutación , Neoplasias/complicaciones , Factores de Transcripción/genética , Anciano , Análisis Mutacional de ADN , Dermatomiositis/etiología , Dermatomiositis/metabolismo , Femenino , Humanos , Masculino , Factores de Transcripción/metabolismo , Dedos de ZincRESUMEN
Background: Certain associated and specific myositis antibodies are related to certain clinical phenotypes of dermatomyositis (DM), disease severity and the presence of cancer. Aim: To describe the clinical profile of Chilean patients with DM and their associated and specific myositis antibodies. Material and Methods: Review of medical records of 15 patients with DM aged 31 to 72 years. Their clinical characteristics, laboratory tests and complementary tests were reviewed. In serum samples from each patient the presence of 16 specific antibodies was analyzed by immunoblot technique (Myositis Profile Euroline Blot test kit). Results: Fourteen (93.3%) patients had skin manifestations, five (33.3%) had pulmonary involvement, two (13.3%) had an associated cancer and nine (60%) had specific antibodies associated with myositis. Conclusions: These patients with DM had a clinical profile similar to what has been described elsewhere. The profile of myositis specific antibodies was different from reports in other populations.
Asunto(s)
Humanos , Masculino , Femenino , Persona de Mediana Edad , Anciano , Autoanticuerpos/sangre , Dermatomiositis/diagnóstico , Piel/inmunología , Piel/patología , Autoanticuerpos/inmunología , Dermatomiositis/etiología , Dermatomiositis/sangreRESUMEN
La dermatomiositis (DM) es una miopatía inflamatoria de causa desconocida caracterizada por inflamación muscular, debilidad músculo-esquelética proximal y manifestaciones cutáneas típicas. Se ha asociado a malignidades como un síndrome paraneoplásico. Reportamos el caso de un paciente varón de 33 años, diagnosticado de hepatitis B, VHB crónico inactivo, que presentó lesiones papulares, pruriginosas y descamativas en cara, manos, zona inguinal y pies. Al examen físico se evidenció pápulas de Gottron, signo del heliotropo, debilidad muscular simétrica proximal. Se realizó una biopsia de piel donde se encontraron hallazgos compatibles con DM. Tras una ecografía abdominal se encontró una tumoración hepática, cuyo resultado en biopsia fue de carcinoma hepatocelular moderadamente diferenciado. Posteriormente se le realiza segmentectomía con lo cual síntomas de DM disminuyen. Es un caso infrecuente, y de sumo interés por lo que se decide reportar.
Dermatomyositis is an idiopathic inflammatory myopathie characterized by proximal skeletal muscle weakness, typical skin manifestations and muscle inflammation. This disease has been associated with malignancies as a paraneoplastic syndrome. We present a patient of thirty-three years diagnosed with hepatitis B, chronic inactive HBV who presents papular, pruritic and desquamative lesions on the face, hands, inguinal area and feet. At the physical examination is evidentiated Gottron's papules, heliotrope sign and proximal symmetric muscular weakness. Findings compatible with DM were found in a skin biopsy. An abdominal ultrasound revealed a liver tumor whereby a biopsy was performed and the result was a moderately differentiated hepatocellular carcinoma. Subsequently, a segmentectomy has been made and consequently the DM symptoms decreased. This case is of great interest and rare reason why we decided to reported it.
Asunto(s)
Adulto , Humanos , Masculino , Virus de la Hepatitis B , Carcinoma Hepatocelular/complicaciones , Hepatitis B Crónica/complicaciones , Dermatomiositis/etiología , Neoplasias Hepáticas/complicaciones , Perú , Piel/patología , Carcinoma Hepatocelular/virología , Carcinoma Hepatocelular/diagnóstico por imagen , Dermatomiositis/patología , Neoplasias Hepáticas/virología , Neoplasias Hepáticas/diagnóstico por imagenAsunto(s)
Adenocarcinoma/congénito , Neoplasias del Colon/complicaciones , Dermatomiositis/diagnóstico , Dermatomiositis/etiología , Exantema/etiología , Hemorragia Gastrointestinal/etiología , Hepatitis B Crónica/complicaciones , Humanos , Masculino , Persona de Mediana Edad , Debilidad Muscular/etiologíaRESUMEN
Dermatomyositis is an idiopathic inflammatory myopathie characterized by proximal skeletal muscle weakness, typical skin manifestations and muscle inflammation. This disease has been associated with malignancies as a paraneoplastic syndrome. We present a patient of thirty-three years diagnosed with hepatitis B, chronic inactive HBV who presents papular, pruritic and desquamative lesions on the face, hands, inguinal area and feet. At the physical examination is evidentiated Gottron's papules, heliotrope sign and proximal symmetric muscular weakness. Findings compatible with DM were found in a skin biopsy. An abdominal ultrasound revealed a liver tumor whereby a biopsy was performed and the result was a moderately differentiated hepatocellular carcinoma. Subsequently, a segmentectomy has been made and consequently the DM symptoms decreased. This case is of great interest and rare reason why we decided to reported it.
Asunto(s)
Carcinoma Hepatocelular/complicaciones , Dermatomiositis/etiología , Virus de la Hepatitis B , Hepatitis B Crónica/complicaciones , Neoplasias Hepáticas/complicaciones , Adulto , Carcinoma Hepatocelular/diagnóstico por imagen , Carcinoma Hepatocelular/virología , Dermatomiositis/patología , Humanos , Neoplasias Hepáticas/diagnóstico por imagen , Neoplasias Hepáticas/virología , Masculino , Perú , Piel/patologíaRESUMEN
Dermatomyositis is an autoimmune inflammatory myopathy associated with dermatologic findings. Its association with neoplasms is widely reported and has been shown to be higher in lung cancer, particularly small-cell carcinoma, probably as it originates from neuroendocrine cell precursors, as well as breast and ovarian cancer. The low frequency of dermatomyositis makes it difficult to suspect the underlying lesions among the diagnostic possibilities of systemic diseases with cutaneous manifestations. Therefore, the high probability of a neoplasm should always be considered, with reports of up to 75% in the eastern population and about 40% in general. In this report, we present a rare case of dermatomyositis with lesions in a shawl-like distribution, as a symptomatic debut of a small cell lung carcinoma.
La dermatomiositis es una miopatía inflamatoria autoinmune, asociada a manifestaciones dermatológicas. Su vinculación con neoplasias está ampliamente reportada, siendo más común en el cáncer pulmonar, especialmente en carcinoma de células pequeñas probablemente por su origen en precursores de células neuroendocrinas, así como también en cáncer de mama y ovario. La baja frecuencia de dermatomiositis dificulta la sospecha de las lesiones subyacentes entre las posibilidades diagnósticas de las enfermedades sistémicas con manifestaciones cutáneas. Por ello siempre se debe considerar la alta probabilidad de una neoplasia, con reportes de hasta 75% en la población oriental y cerca de 40% en general. En este artículo se presenta un caso poco frecuente de dermatomiositis con lesiones de distribución en chal, como debut sintomático de un carcinoma pulmonar de células pequeñas.
Asunto(s)
Dermatomiositis/etiología , Neoplasias Pulmonares/diagnóstico , Síndromes Paraneoplásicos/etiología , Carcinoma Pulmonar de Células Pequeñas/diagnóstico , Dermatomiositis/patología , Humanos , Neoplasias Pulmonares/complicaciones , Masculino , Persona de Mediana Edad , Síndromes Paraneoplásicos/patología , Carcinoma Pulmonar de Células Pequeñas/complicacionesRESUMEN
Paraneoplastic disorders are manifestations of internal malignancies without the direct action of the tumor. Its pathogenesis involves production of substances that interfere with cellular activity of distant tissues. Paraneoplasias may be the first sign of cancer, and clinicians should be familiarized with its manifestations in order to perform an early diagnosis of the underlying neoplasm. The aim of this review was to describe most common paraneoplastic skin diseases.
Asunto(s)
Síndromes Paraneoplásicos/complicaciones , Síndromes Paraneoplásicos/diagnóstico , Enfermedades de la Piel/diagnóstico , Enfermedades de la Piel/etiología , Acantosis Nigricans/diagnóstico , Acantosis Nigricans/etiología , Índice de Masa Corporal , Brasil/epidemiología , Dermatitis Exfoliativa/diagnóstico , Dermatitis Exfoliativa/etiología , Dermatomiositis/diagnóstico , Dermatomiositis/etiología , Detección Precoz del Cáncer , Humanos , Hipertricosis/diagnóstico , Hipertricosis/etiología , Resistencia a la Insulina , Neoplasias/complicaciones , Neoplasias/diagnóstico , Obesidad/complicaciones , Síndromes Paraneoplásicos/epidemiología , Factores de Riesgo , Enfermedades de la Piel/epidemiologíaRESUMEN
BACKGROUND: Dermatomyositis and polymyositis are both types of idiopathic inflammatory myositis characterized by inflammation and weakness of proximal skeletal muscles and skin rash. CASE: A 49-year-old Caucasian woman recently diagnosed with breast cancer classified as T1N2M0, stage IIIA, presenting skin rash associated with heliotrope and Gottron's papules. In addition, there was a progression to a severe reduction in proximal muscle strength with severe dysphagia. The initial treatment was conducted, and the patient recovered from all symptoms and followed adjuvant cancer management. TREATMENT: At first, high dose of corticosteroid was administered as pulse therapy, and a radical mastectomy was indicated due to the severe symptoms of the paraneoplastic syndrome. Then chemotherapy and radiotherapy were applied, and oral corticoid associated with immunosupressive drug was administered for dermatomyositis control. DISCUSSION: The association between myositis and an increased risk of cancer has been demonstrated over the years. This patient has a high probability of dermatomyositis diagnosis. The initial treatment with high dose of glucocorticoids may result in an improvement of muscle lesions. Second-line treatment with azathioprine, methotrexate, or cyclophosphamide may be required for aggressive disease. Removal of the cancer induces improvement of paraneoplastic syndrome. CONCLUSION: Dermatomyositis can be a clinical manifestation of a paraneoplastic syndrome in patients with breast cancer. It is a rare diagnosis, and there is little evidence to guide treatment until now. It is possible to control the evolution of dermatomyositis with high doses of glucocorticoids in almost all cases; however, in severe cases of paraneoplastic syndrome, cancer treatment should start immediately.
Asunto(s)
Neoplasias de la Mama/complicaciones , Dermatomiositis/diagnóstico , Síndromes Paraneoplásicos/diagnóstico , Polimiositis/diagnóstico , Neoplasias de la Mama/patología , Dermatomiositis/etiología , Dermatomiositis/terapia , Diagnóstico Diferencial , Femenino , Humanos , Persona de Mediana Edad , Síndromes Paraneoplásicos/etiología , Síndromes Paraneoplásicos/terapia , Polimiositis/etiología , Polimiositis/terapia , PronósticoRESUMEN
OBJECTIVE: To evaluate the influence of exposure to inhaled environmental factors during pregnancy on the diagnosis of juvenile dermatomyositis (DM). METHODS: We performed a casecontrol study comprising 20 juvenile DM patients and 56 healthy controls matched by age and sex who were residents in the metropolitan region of a large city. A questionnaire assessed demographic data and environmental inhalation exposure during pregnancy (occupational exposure to demolition, chalk, construction and/or quarry dust, paints, varnish, gasoline vapor, and/or battery fluids; stationary sources of inhaled pollution near the mother's home; and maternal tobacco exposure). Daily concentrations of inhaled particulate matter, SO2, NO2, O3, and carbon monoxide (CO) were evaluated throughout the gestational period. RESULTS: Maternal occupational exposure to school chalk dust/gasoline vapor in the juvenile DM group was significantly higher compared with controls (50% versus 4.6%; P = 0.001). Smoking mothers and secondhand smoke exposure at home during pregnancy were significantly higher in the juvenile DM group versus controls (smoking mothers: 20% versus 1.7%; P = 0.01, and secondhand smoke: 35% versus 19%; P = 0.07). In univariate logistic regression models, maternal smoking, occupational exposure to inhaled agents, and the highest tertile of tropospheric CO (3.25.4 parts per million) in the third trimester were significantly associated with juvenile DM (P ≤ 0.05). In the multivariate analysis, smoking mother (odds ratio [OR] 13.26 [95% confidence interval (95% CI) 1.21144.29], P = 0.03), occupational exposure (OR 35.39 [95% CI 1.97632.80], P = 0.01), and CO (third tertile) exposure in the third trimester of gestation (OR 12.21 [95% CI 1.28115.96], P = 0.03) remained risk factors for juvenile DM. CONCLUSION: Inhaled pollutants and tobacco smoking during fetal development may contribute to juvenile DM.
Asunto(s)
Contaminantes Ocupacionales del Aire/efectos adversos , Dermatomiositis/etiología , Exposición por Inhalación/efectos adversos , Exposición Materna/efectos adversos , Exposición Profesional/efectos adversos , Efectos Tardíos de la Exposición Prenatal , Fumar/efectos adversos , Contaminación por Humo de Tabaco/efectos adversos , Adolescente , Brasil , Monóxido de Carbono/efectos adversos , Estudios de Casos y Controles , Distribución de Chi-Cuadrado , Niño , Preescolar , Dermatomiositis/diagnóstico , Femenino , Edad Gestacional , Humanos , Lactante , Modelos Logísticos , Masculino , Análisis Multivariante , Oportunidad Relativa , Embarazo , Factores de Riesgo , Encuestas y Cuestionarios , Salud UrbanaRESUMEN
Infective endocarditis (IE) may present with rheumatological manifestations such as myalgias, arthralgias, arthritis and back pain. However, muscle inflammation is rare. We present a case of a 68-year-old Hispanic man who presented with 1-month history of tiredness, weight loss, fever, myalgias, muscle weakness and dysphagia to solid food. On physical examination he had severe weakness in the proximal upper and lower extremities, and erythematous eruption involving the upper eyelids, neck and metacarpophalangeal joints. Creatine kinase levels were markedly elevated at 15 809 U/L. MRI of the right thigh revealed intermuscular and intramuscular oedema. Muscle biopsy showed acute necrotising suppurative perimyositis. Blood cultures were positive for methicillin-resistant Staphylococcus aureus. A transoesophageal echocardiogram revealed vegetations in the pulmonic valve. All clinical manifestations were resolved completely with broad-spectrum antibiotics. This case suggests that IE should be considered in the differential diagnosis of a patient presenting with inflammatory myopathy.
Asunto(s)
Dermatomiositis/diagnóstico , Endocarditis/diagnóstico , Infecciones Estafilocócicas , Anciano , Bacteriemia/microbiología , Trastornos de Deglución/microbiología , Dermatomiositis/etiología , Diagnóstico Diferencial , Endocarditis/complicaciones , Endocarditis/microbiología , Fatiga/microbiología , Fiebre/microbiología , Humanos , Masculino , Debilidad Muscular/microbiología , Mialgia/microbiología , Miositis/microbiología , Staphylococcus aureus , Pérdida de PesoRESUMEN
OBJECTIVE: Dermatomyositis (DM) symptoms may be a clue to the existence of a hidden cancer. Enhancing early detection is essential, but there are no studies evaluating short-term predictive factors in this disease. METHODS: This is a single-center retrospective study, including patients diagnosed with DM meeting at least four of the five Bohan and Peter's criteria (1975), from 1991 to 2011. This study assessed malignancies occurring in up to 12 months after the diagnosis of DM. RESULTS: Neoplasm was found in 12 out of 139 patients (skin, gastrointestinal tract, prostate, thyroid, breast, lungs, and genitourinary tract). Patients with neoplasm had a higher mean age than controls (56.8 ± 15.7 vs. 40.3 ± 13.1 years, respectively, P = 0.004, odds ratio 1.09; 95% confidence interval: 1.04-1.14). No statistical differences were observed regarding gender, ethnicity, frequency of constitutional symptoms, organ and systemic involvements, and/or laboratory alterations. CONCLUSION: In newly diagnosed DM, age at disease diagnosis was a predictive factor of malignancy.
Asunto(s)
Dermatomiositis/diagnóstico , Neoplasias/epidemiología , Adulto , Anciano , Anciano de 80 o más Años , Dermatomiositis/etiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Neoplasias/complicaciones , Pronóstico , Estudios Retrospectivos , Adulto JovenRESUMEN
OBJETIVO: Os sintomas da dermatomiosite (DM) podem ser um indício da existência de um câncer oculto. Melhorar a detecção precoce é essencial, porém não há estudos avaliando em curto prazo os fatores preditivos para a doença. MÉTODO: Estudo retrospectivo, monocêntrico, incluindo pacientes com DM definida (pelo menos quatro dos cinco critérios de Bohan e Peter, 1975), no período entre 1991 e 2011. A presença de malignidade foi limitada a um período de até 12 meses após o diagnóstico da doença. RESULTADOS: Houve 12 casos de neoplasias em 139 pacientes (pele, trato gastrintestinal, próstata, tireoide, mama, pulmão e trato geniturinário). Os pacientes com neoplasia tiveram maior média de idade que os controles (56,8 ± 15,7 vs. 40,3 ± 13,1 anos, respectivamente, P = 0,004; odds ratio 1,09; intervalo de confiança de 95%: 1,04-1,14). Não foram observadas diferenças estatísticas em relação a gênero, etnia, frequência de sintomas constitucionais, envolvimento de órgãos e sistemas e/ou alterações laboratoriais. CONCLUSÃO: Na DM recém-diagnosticada, a idade tardia ao diagnóstico foi um fator preditivo de malignidade.
OBJECTIVE: Dermatomyositis (DM) symptoms may be a clue to the existence of a hidden cancer. Enhancing early detection is essential, but there are no studies evaluating short-term predictive factors in this disease. METHODS: This is a singlecenter retrospective study, including patients diagnosed with DM meeting at least four of the five Bohan and Peter's criteria (1975), from 1991 to 2011. This study assessed malignancies occurring in up to 12 months after the diagnosis of DM. RESULTS: Neoplasm was found in 12 out of 139 patients (skin, gastrointestinal tract, prostate, thyroid, breast, lungs, and genitourinary tract). Patients with neoplasm had a higher mean age than controls (56.8 ± 15.7 vs. 40.3 ± 13.1 years, respectively, P = 0.004, odds ratio 1.09; 95% confidence interval: 1.04-1.14). No statistical differences were observed regarding gender, ethnicity, frequency of constitutional symptoms, organ and systemic involvements, and/or laboratory alterations. CONCLUSION: In newly diagnosed DM, age at disease diagnosis was a predictive factor of malignancy.
Asunto(s)
Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , Dermatomiositis/diagnóstico , Neoplasias/epidemiología , Dermatomiositis/etiología , Neoplasias/complicaciones , Pronóstico , Estudios RetrospectivosRESUMEN
A dermatomiosite é uma miopatia inflamatória que cursa com manifestações cutâneas. No adulto acima de 50 anos, pode estar associada a neoplasias malignas, comportando-se como um grande sinalizador de malignidade. OBJETIVO: Demonstrar essa associação com aspectos peculiares: dermatomiosite de apresentação atípica paralela a dois tumores sincrônicos, em geral, não relacionados àquela. RELATO DO CASO: Descrevemos o caso de um paciente do gênero masculino de 72 anos, que desenvolveu dermatomiosite, inicialmente apenas com quadro cutâneo clássico, evoluindo com lesões vesiculobolhosas, e, meses após, com miopatia. Após extensa investigação, foi localizado um adenocarcinoma de próstata. Houve remissão da doença após tratamento do câncer e administração de glicocorticoide. Durante a retirada gradual do glicocorticoide, ocorreu reativação da dermatomiosite, sendo reiniciadas investigações, que revelaram a presença de carcinoma escamoso de língua. Tratada esta neoplasia, houve remissão completa, mesmo após a retirada total do corticoide. CONCLUSÃO: Trata-se de um caso raro por envolver as formas menos usuais de apresentação da dermatomiosite, tanto com relação ao quadro cutaneomuscular, quanto à sua associação a tumores de próstata e língua (tumores nunca antes relatados juntos). Este caso demonstra a importância da investigação minuciosa em busca de neoplasias na abordagem desses pacientes.
Dermatomyositis is an inflammatory myopathy with skin manifestations. In the adult over the age of 50 years, it can be associated with malignant neoplasias, being, thus, a signal of malignancy. OBJECTIVE: To show the association of dermatomyositis of atypical presentation with two synchronous tumors, usually not related to that. CASE REPORT: We report the case of a 72-year-old male, who developed dermatomyositis, initially with only classic skin findings, which progressed to vesiculobullous lesions, and, months later, to myopathy. After extensive investigation, prostate adenocarcinoma was diagnosed. After treatment of the cancer and administration of glucocorticoid, the disease went into remission. During gradual glucocorticoid withdrawal, dermatomyositis recurred, and the new investigation revealed the presence of squamous cell carcinoma of the tongue. After treating this neoplasia, complete remission occurred, even after total corticoid withdrawal. CONCLUSION: This is a rare case involving less usual dermatomyositis presentation forms, relating to the cutaneous-muscle findings and the association with prostate and tongue tumors (tumors never reported together). This case demonstrates the importance of a careful investigation, searching for neoplasias, when approaching such patients.
Asunto(s)
Anciano , Humanos , Masculino , Dermatomiositis/etiología , Neoplasias Primarias Múltiples/complicaciones , Síndromes Paraneoplásicos/complicaciones , Neoplasias de la Próstata/complicaciones , Enfermedades Cutáneas Vesiculoampollosas/etiología , Neoplasias de la Lengua/complicacionesRESUMEN
UNLABELLED: Dermatomyositis is an inflammatory myopathy with skin manifestations. In the adult over the age of 50 years, it can be associated with malignant neoplasias, being, thus, a signal of malignancy. OBJECTIVE: To show the association of dermatomyositis of atypical presentation with two synchronous tumors, usually not related to that. CASE REPORT: We report the case of a 72-year-old male, who developed dermatomyositis, initially with only classic skin findings, which progressed to vesiculobullous lesions, and, months later, to myopathy. After extensive investigation, prostate adenocarcinoma was diagnosed. After treatment of the cancer and administration of glucocorticoid, the disease went into remission. During gradual glucocorticoid withdrawal, dermatomyositis recurred, and the new investigation revealed the presence of squamous cell carcinoma of the tongue. After treating this neoplasia, complete remission occurred, even after total corticoid withdrawal. CONCLUSION: This is a rare case involving less usual dermatomyositis presentation forms, relating to the cutaneous-muscle findings and the association with prostate and tongue tumors (tumors never reported together). This case demonstrates the importance of a careful investigation, searching for neoplasias, when approaching such patients.
Asunto(s)
Dermatomiositis/etiología , Neoplasias Primarias Múltiples/complicaciones , Síndromes Paraneoplásicos/complicaciones , Neoplasias de la Próstata/complicaciones , Enfermedades Cutáneas Vesiculoampollosas/etiología , Neoplasias de la Lengua/complicaciones , Anciano , Humanos , MasculinoRESUMEN
Juvenile dermatomyositis (JDM) is an autoimmune disease characterized by systemic vasculopathy. Its main manifestations include symmetrical proximal muscle weakness, elevated serum muscle enzymes and cutaneous lesions, among which the heliotrope and Gottron's papules are pathognomonic. Early recognition and prompt therapy allow better prognosis and prevent the development of calcinosis. Although the treatment is based on glucocorticoids, the more commonly associated immunosuppressors include methotrexate, azathioprine, cyclosporine, and cyclophosphamide, depending on the severity of disease. The use of immunobiologicals for refractory cases remains under investigation, but the results are controversial or inexpressive. In this review, we highlight recent updates on the pathogenesis and treatment of JDM.
Asunto(s)
Dermatomiositis , Dermatomiositis/diagnóstico , Dermatomiositis/tratamiento farmacológico , Dermatomiositis/etiología , HumanosRESUMEN
JUSTIFICATIVA E OBJETIVOS: A dermatomiosite é umadoença sistêmica crônica, de etiologia desconhecida, que se caracterizapor acometimento inflamatório da pele e dos músculos.O diagnóstico baseia-se nos achados clínicos e laboratoriais. Os corticoides são a terapia mais utilizada. As causas de óbito maisfrequentes são neoplasia maligna, septicemia e fibrose pulmonar.O objetivo deste estudo foi efetuar uma ampla revisão de literatura com foco no reconhecimento dos principais achados clínicos e no tratamento desta doença.CONTEÚDO: A dermatomiosite é uma doença sistêmica crônica que se caracteriza por acometimento inflamatório da pele e dos músculos. Possui duas formas principais: miopática, maisfrequente, onde se encontram lesões musculares e cutâneas; e amiopática, somente com lesões cutâneas. O sexo feminino é o mais afetado, e a idade média do diagnóstico é 40 anos. Manifestações cutâneas são observadas em todos os pacientes. Das alterações sistêmicas, a manifestação muscular mais frequente é a perda de força proximal, e a manifestação pulmonar mais comum é a pneumopatia intersticial. Podem ser observadas neoplasias durante o seguimento da doença, sendo mais frequentes nos pacientes acima de 60 anos. A desidrogenase lática é a enzima muscular alterada na maioria dos casos. O diagnóstico da dermatomiositepode ser realizado por exame anatomopatológico de biópsia muscular,além de eletroneuromiografia. Os corticoides são os mais utilizados. As causas de óbito mais frequentes são neoplasia maligna,septicemia e fibrose pulmonar. Não há causa conhecida. Odiagnóstico baseia-se nos achados clínicos e laboratoriais.CONCLUSÃO: Através da analise dos dados bibliográficos foi possível concluir que a dermatomiosite é uma doença de diagnóstico predominantemente clínico. Os exames laboratoriais e de imagem constituem importantes confirmadores do quadro, mas nunca são identificadores isolados, sendo sempre a clínica, soberana no diagnóstico desta doença.(...)
BACKGROUND AND OBJECTIVES: The dermatomyosits is a systemic chronic disease, of unknown etiology, which is characterized for inflammatory attack of the skin and muscles. The diagnosis is based on clinical and laboratory finds. The corticosteroids are the most used therapy. The most frequent causes of death are: the malignant neoplasia, the septicemia and the pulmonary fibrosis. A bibliographical revision was carried out, with the objective of attracting attention of the doctors for the recognition of the principal clinical finds of this illness. CONTENTS: The dermatomyositis is a systemic chronic disease that is characterized by the inflammatory attack of the skin and muscles. It has two principal forms: miopatic that is more frequent, and presents muscular and skin injuries; and the amiopatic that presents skin injuries only. The feminine sex is more affected mainly at the middle age of 40s. Skin manifestations are observed in all the patients. Among the systemic alterations,the most frequent muscular demonstration is the loss of strength proximal, and the commonest pulmonary demonstration is interstitial pneumopathy. Neoplasis can be observed during the continuation of the disease, and is more frequent in the patients above 60 years old. The Lactic dehydrogenase means that the muscular enzyme is altered in most of the cases. The diagnosis of the dermatomyositis can be done for the muscular biopsy applyinganatomopatologic exam, besides electroneuromiography examination. The corticosteroids are the most used therapy. The most frequent causes of death are the malignant neoplasis, sepsis, and the pulmonary fibrosis and there is no known cause. The diagnosis is based on the clinical and laboratories finds.CONCLUSION: Through the analyzes of the bibliographical facts it is possible to conclude that dermatomiositis is a disease of predominantly clinical diagnosis.(...)
Asunto(s)
Humanos , Dermatomiositis/epidemiología , Dermatomiositis/etiología , Dermatomiositis/patología , MiositisRESUMEN
In SLE, scleroderma, and PM/DM, infections are important causes of morbidity and mortality. This increased risk of developing infections is the result of immune abnormalities and of organ system manifestations associated with these diseases and their treatments. Common bacteria are responsible for most mild and lethal infections; however, opportunistic microorganisms cause death in some patients, particularly in those receiving high doses of corticosteroid and immunosuppressive therapy. Various viral and fungal infections also contribute to the morbidity and mortality associated with these diseases. Regardless of the cause of infections, adequate and prompt recognition and proper treatment of the infected patient are imperative. Thus, patients with these diseases, especially when receiving high doses of corticosteroids and immunosuppressive therapy, need to be monitored closely for these infections. This care and concern is necessary to ensure optimal patient outcomes, both in terms of morbidity and mortality.