ABSTRACT
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.
Subject(s)
Dermatomyositis/epidemiology , Paraneoplastic Syndromes/epidemiology , Adolescent , Adult , Aged , Aged, 80 and over , Carcinoma , Confidence Intervals , Dermatomyositis/complications , Dermatomyositis/immunology , Dermatomyositis/pathology , Female , Humans , Logistic Models , Male , Middle Aged , Muscle Weakness , Nasopharyngeal Carcinoma , Nasopharyngeal Neoplasms/epidemiology , Nasopharyngeal Neoplasms/immunology , Nasopharyngeal Neoplasms/pathology , Odds Ratio , Paraneoplastic Syndromes/complications , Paraneoplastic Syndromes/immunology , Paraneoplastic Syndromes/pathology , Retrospective Studies , Risk Factors , Singapore/epidemiology , Young AdultABSTRACT
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.
Subject(s)
Autoantibodies/blood , Enzyme-Linked Immunosorbent Assay/methods , Fluorescent Antibody Technique, Indirect/methods , Pemphigus/blood , Pemphigus/diagnosis , Animals , Biomarkers/blood , Dermatology/instrumentation , Dermatology/methods , Desmogleins/metabolism , Enzyme-Linked Immunosorbent Assay/instrumentation , Esophagus/chemistry , Esophagus/pathology , Fluorescent Antibody Technique, Indirect/instrumentation , Haplorhini , Humans , Sensitivity and Specificity , Skin/chemistry , Skin/pathology , Substrate SpecificitySubject(s)
Asian People , Dermatomyositis/etiology , Nasopharyngeal Neoplasms/complications , Paraneoplastic Syndromes/etiology , Dermatomyositis/ethnology , Dermatomyositis/pathology , Female , Humans , Middle Aged , Nasopharyngeal Neoplasms/diagnosis , Nasopharyngeal Neoplasms/ethnology , Paraneoplastic Syndromes/ethnology , Paraneoplastic Syndromes/pathology , Skin/pathologyABSTRACT
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.
Subject(s)
DNA, Viral/genetics , Erythema Multiforme/complications , Herpes Simplex/complications , Simplexvirus/isolation & purification , Skin/pathology , Biopsy , Humans , Polymerase Chain Reaction , Simplexvirus/geneticsABSTRACT
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.