RESUMO
INTRODUCTION: Dermatomyositis (DM) is a multisystem inflammatory disease with a strong association with malignancy. We aimed to describe a series of Asian patients with DM and identify any significant clinical factors associated with malignancy. MATERIALS AND METHODS: This was a retrospective review of a multi-racial cohort of 69 Asian patients diagnosed with DM over an 11-year period from 1996 to 2006. RESULTS: Malignancy was detected in 15 out of 68 patients (22%), the most common of which was nasopharyngeal carcinoma (7 cases). Compared to the non-malignancy group, the malignancy-associated group was older and had more male patients. There were no statistically significant clinical, serological or laboratory factors associated with a higher risk of malignancy. CONCLUSION: This study highlights the importance of ongoing malignancy screening especially for nasopharyngeal carcinoma in Asian patients with DM.
Assuntos
Dermatomiosite/epidemiologia , Síndromes Paraneoplásicas/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinoma , Intervalos de Confiança , Dermatomiosite/complicações , Dermatomiosite/imunologia , Dermatomiosite/patologia , Feminino , Humanos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Debilidade Muscular , Carcinoma Nasofaríngeo , Neoplasias Nasofaríngeas/epidemiologia , Neoplasias Nasofaríngeas/imunologia , Neoplasias Nasofaríngeas/patologia , Razão de Chances , Síndromes Paraneoplásicas/complicações , Síndromes Paraneoplásicas/imunologia , Síndromes Paraneoplásicas/patologia , Estudos Retrospectivos , Fatores de Risco , Singapura/epidemiologia , Adulto JovemAssuntos
Povo Asiático , Dermatomiosite/etiologia , Neoplasias Nasofaríngeas/complicações , Síndromes Paraneoplásicas/etiologia , Dermatomiosite/etnologia , Dermatomiosite/patologia , Feminino , Humanos , Pessoa de Meia-Idade , Neoplasias Nasofaríngeas/diagnóstico , Neoplasias Nasofaríngeas/etnologia , Síndromes Paraneoplásicas/etnologia , Síndromes Paraneoplásicas/patologia , Pele/patologiaRESUMO
A prospective study was performed to assess the usefulness of desmoglein enzyme-linked immunosorbent assay testing compared with indirect immunofluorescence in the diagnosis of new cases of pemphigus, as well as to compare the relative sensitivities of monkey oesophagus and normal human skin as substrates for indirect immunofluorescence. These tests were performed on the sera of 29 consecutive new cases of pemphigus diagnosed over a 2-year period based on clinical, histological and direct immunofluorescence findings. Desmoglein enzyme-linked immunosorbent assay was positive in all patients whereas indirect immunofluorescence was positive in only 25 of 29 patients. All four patients with negative indirect immunofluorescence had positive antinuclear antibodies or cytoplasmic fluorescence that could have masked the anti-intercellular antibodies. Desmoglein enzyme-linked immunosorbent assay appeared to reflect the disease activity better than indirect immunofluorescence in a few patients who had active disease of recent onset. Monkey oesophagus was found to be superior or equal to human skin as a substrate for indirect immunofluorescence in both pemphigus vulgaris and foliaceus.
Assuntos
Autoanticorpos/sangue , Ensaio de Imunoadsorção Enzimática/métodos , Técnica Indireta de Fluorescência para Anticorpo/métodos , Pênfigo/sangue , Pênfigo/diagnóstico , Animais , Biomarcadores/sangue , Dermatologia/instrumentação , Dermatologia/métodos , Desmogleínas/metabolismo , Ensaio de Imunoadsorção Enzimática/instrumentação , Esôfago/química , Esôfago/patologia , Técnica Indireta de Fluorescência para Anticorpo/instrumentação , Haplorrinos , Humanos , Sensibilidade e Especificidade , Pele/química , Pele/patologia , Especificidade por SubstratoRESUMO
BACKGROUND AND OBJECTIVE: We report on a 42-year-old man with Stage IIA non-Hodgkin's lymphoma who developed a severe mucocutaneous blistering eruption. His diagnosis, paraneoplastic pemphigus, was based on clinical, histological, and immunofluorescence findings and confirmed by immunoprecipitation. Despite maximal therapy with plasmapheresis, corticosteroids, and mycophenolate mofetil and the subsequent addition of cyclophosphamide and cyclosporine, the condition was fatal. CONCLUSION: This case illustrates the refractory nature of this disease and the inadequacy of existing therapies.
Assuntos
Linfoma não Hodgkin/etiologia , Síndromes Paraneoplásicas/etiologia , Pênfigo/complicações , Corticosteroides/uso terapêutico , Adulto , Antibióticos Antineoplásicos/uso terapêutico , Ciclofosfamida/uso terapêutico , Ciclosporina/uso terapêutico , Evolução Fatal , Humanos , Imunossupressores/uso terapêutico , Masculino , Ácido Micofenólico/uso terapêutico , Síndromes Paraneoplásicas/tratamento farmacológico , Síndromes Paraneoplásicas/patologia , PlasmafereseRESUMO
BACKGROUND: The wide variation in the detection of herpes simplex virus (HSV) DNA (36-75%) by polymerase chain reaction (PCR) in erythema multiforme (EM) may be partly attributed to differences in case selection in terms of subsets of EM studied. OBJECTIVE: To determine the frequencies of detection of HSV DNA in specific subsets of EM. METHODS: Nested PCR was used to detect HSV DNA in skin biopsies with histologically proven EM. RESULTS: PCR was performed on skin biopsies from 63 patients with EM. HSV DNA was detected in 3/11 (27.2%) of single-episode HSV-associated EM (HAEM), 6/10 (60%) of recurrent HAEM, 1/4 (25%) of single-episode idiopathic EM and 6/12 (50%) of recurrent idiopathic EM. HSV DNA was not detected in atypical EM (0/11), suspected drug-induced EM (0/9) or Stevens-Johnson syndrome (0/6). CONCLUSION: The overall PCR positive rates of HAEM (42.9%) and idiopathic EM (43.8%) were comparable suggesting that idiopathic EM is likely to be related to a subclinical HSV infection.