The Analysis Of Clinic And Pathyology For30Cases With Dermatomyositis

Objective: The purpose of this study was to summarize and analyse theclinical, laboratory and myopathological features of dermatomyositis(DM),and to investigate the clinical and pathological features of DM.Methods: We had collected the clinical data of80patients who werediagnosed inflammatory myopathy from2004to2012in the department ofneuromuscular disease of the Third Hospital of Hebei Medical University.Following the diagnostic criteria of the International Conference of the2004European Neuromuscular Disease Center(ENMC), we chosed30cases DMconcluding confirmed DM(20cases), probable DM (4cases), possibleDM cases(1case), suspicious non-rash DM(5cases). This study reviewedthese data. We undertook open biceps brachii or quadriceps femoris biopsy in30patiens after anesthesia and they all signed the consents. The frozen tissues(7um)were stained with haematoxylin-eosin(HE), modified gomori'strichrome (MGT), nicotinanide adenine dinucleotide(NADH-TR), periodicacid schiff (PAS), oil red O(Oil), acid phosphataseand(Acid), adenosinetriphosphatase(ATPase)and were received pathological analysis under opticalmicroscope. The30cases were divided into three groups according to16years.They were juvenile DM, adult DM and overlap myositis.Results:1General clinical data(Table1,2)The sexual prevalence was1:2. JDM:5females and3males; Adult DM:11females and6males; Overlap syndrome(OM):1male and4female; DM:20female and10male. Mean age:6.5years old of JDM;39.8of adult DM;59.7of adult DM associated with tumor;50of overlap syndrome.Proximal limb weakness mainly accounted for90%(27/30cases),significantly higher than the distal limb weakness; neck muscle weaknessaccounted for60%(8/30cases); muscle pain and/or muscle grip pain accounted for73.33%(22/30cases); throat muscles weakness involved twocases who were adult DM. Abdominal pain was one syndrome that we can notexplain, especially in JDM for100%.Characteristic rash included Heliotrope rash and Gottron rash(66-70%).Adult DM group was higher than the other two groups. The group of OMpatients had Gottron sign(100%); V sign was significantly higher than shawlsign. Three cases of adult DM had holster sign. The skin necrosis or ulcerappeared in three cases of adult DM and two cases of JDM. One JDM and twoadult DM showed calcium deposition in soft tissue. The prognosis weredifferent: JDM(62.5%); OM(40%); adult DM(29.41%). DM with anemia:OM(80%); JDM(75%); adult DM(47%). ESR increased in24cases(80%), also in CK. The anti-the SSA, anti-the SSB, ANA positive appearedin the group of OM. The results of EMG: myopanic chang in27cases;neurogenic abnormalities in1case; normal in2cases.14adult cases hadpulmonary interstitial fibrosis. There was no in JDM.2Pathological analysis of skeletal muscle biopsies(Table3)For all the patients,21cases(70%)had various degrees of muscle fibernecrosis;9cases(30%)showed muscle fibers "punch-out vacuoles";25cases(83.33%)had atrophy fibers in bundle; inflammatory cells infiltrated inbundles of24cases(80%); perivascular inflammatory cell infiltration in29case(s96.67%); small artery walls were thicken in17case(s56.67%); variousdegrees of inflammatory cells infiltrated in fascia included19case(s63.33%);skin biopsy was involved in14cases(46.67%).Conclusion:1IM diagnostic criteria proposed by the European Neuromuscular DiseaseCenter (ENMC2004) contains the features of histochemistry,immunohistochemistry and molecular pathology. The sensitivity of the criteriais low, but accuracy rate is high.The clinical value is the best for DM.2Adult DM has a high incidence of tumors. The risk factors concludeonset-age>45years old, progressive rash especially necrotizing skin lesions,progressive muscle weakness, ESR>35mm/h. 3The biopsy of skeletal muscle, fascia, skin analysis expands pathologicalinformation for diagnosis and differential diagnosis of DM.4The main pathological characters of DM conclude perifascicular atrophies,inflammatory cells around blood vessels of perimysium. The apparent musclefiber necrosis or "ponch-out vacuoles" indicate the severity of illness.5The special markers of inflammatory cells will be the most valuable in theresearch of IM.