Clinical Analysis Of 64 Patients With Pemphigus Vulgaris

Pemphigus is a group life-threatening autoimmune diseases involving the skin,mucous membranes or both.The main character is thin-walled,relaxing and easily-breaked blisters or bullosa.Blisters causedby relaxing of stratum spinosum under the skin are represented on histopathology.Immunopathology showed that IgG,IgA,IgM,or C3 deposit meshily among keratinocytes,and specific anti-keratinocyte substance antibody(also called pemphigus antibody) exist in the serum of patients with various pemphigus.Now it's clear that its target antigen is a transmembrane protein of the structure desmosomes protein. people's own antibodies attack desmosome protein,and the structure of desmosomes happen a series of changes.As a result,it triggers so many clinical manifestations.The anti-epidermal desmosomes antibodies that exist in the serum of Pemphigus patients are mainly IgG.The antibody can cause the loss of cell adhesion,cell lysis,and the formation of blisters under skin or bullosa. Titers of antibody is in parallel with the extent of disease activity.In clinics;most patients manifest pemphigus vulgaris(PV),but there are deciduous type,proliferative,erythema,herpes-like,drug-induced,vice tumors and IgA pemphigus-types and so on.PV is the most common and serious type of pemphigus.Pemphigus mostly occurre in the middle age,and more lesions occurre in the oral mucosa firstly.Skin damages often come forth after repeated oral mucosal injury,which usually appear after 4-6 months.Bullosa can be found firstly in the scalp,arms,back,feet,groin and other places that is vulnerable to chafe,and then blister wall collapse and rupture easily.Following that,a lot of fluid,scars even the offensive stench caused by the secondary infection are general phenomena.The disease are more familiar with peasants and workers,and usually they has been in very serious condition when they are sent to the higher-level hospitals.This is related to the polymorphism of pemphigus,which easily lead to misdiagnosis and delayed treatment,and all this would aggravate the condition.Glucocorticoid is the first choice in the treatment of the disease.In the absence of glucocorticoid treatment,mortality rate of the disease can reach 75%,while the rate of clinical remission of the disease is significantly improved due to glucocorticoid or the use of glucocorticoid and immunosuppressants.There are still a few severe pemphigus patients died of chronic consumption,severe infections,gastrointestinal bleeding or other complications.Although the diagnosis,treatment and prognosis of the disease are more understanding than that in the past,but now the incidence of this disease,the rate of misdiagnosis,complications and mortality have not been paid enough attention to by clinicians so as to cause great harm to public health.64 cases of severe pemphigus patients from October 1997 to October 2007 were analyzed retrospectively in the study,and the incidence of desease in men and women,age of onset,initial lesion site,the rate of misdiagnosis,inducement, morbidity,the relations to patients' professional,efficacy of treatment, accompanied diseases and various complications are analysed comprehensively. Diagnosis of the disease mainly rely on the typical clinical presentation, histopathology and immunopathological examination.64 cases cases compose of 61 cases of pemphigus vulgaris(PV)and 3 cases of pemphigus erythematosus(PE).While 33 male cases and 31 female cases,and the ratio of male and female is 1.06:1.Patients' age of onset is between the ages of 15 to 82, with an average(47.02±15.65)years.Patients of skin involved is 30 cases,mucosal involved is 38 cases,including 29 patients(about 45.3%)of oral mucosal erosion and ulcers as the first site for the performance,10 cases of skin lesions,head for the first,4 cases of the external genitalia for the first,21 cases of the trunk for the first.Meanwhile,patients make up of 35 cases of farmers,21 cases of workers and 8 cases of others.20 cases of patients in 64 cases are misdiagnosed,and rate of misdiagnosis is 31.25%.Besides,two cases of Manchu,two cases of Inner Mongolia,one case of Korean,59 cases of Han.While 21 patients make use of glucocorticoid therapy alone,43 cases of patients with glucocorticoid and immunosuppressant therapy.33 cases of patients in 64 cases are cured,20 cases are noticeably improved,11 patients are improved.Two cases died during follow up.Statistical analysis using SPSS11.0 statistical software.The results of this study take on as follow:The incidence rates of pemphigus in men and women are basically the same,and have no significant differences.The incidence of people from 30 to 50 years old is 51.56%,and that of patients less than 30 years of age accounte for 10.94%.That can be seen from the incidence of the disease has the trend of development to the younger age.20 cases(31.25%)of patients in 64 cases were misdiagnosed as other skin diseases in the pre-hospital.This is because the disease is relatively lower in clinics,then lesions of some patients are polymorphism,and clinicians lack of sufficient knowledge of this disease.35 cases are farmers,21 cases are workers,and 8 cases are others.That means farmers and workers are the majority of patients with pemphigus,which indicates whether relate to their work environment or not.From the perspective of the nation,Manchu two cases,Inner Mongolia two cases,one case of Korean and 59 cases of Han.Whether this show the Han people have the high incidence is worthy to think about.The treatment to a separate is the application of glucocorticoid steroids,and the joint of glucocorticoid and immunosuppressant.Joint immunosuppressant cure rate was higher than that of a separate group of glucocorticoid group,Joint immunosuppressant lesions control group was significantly shorter than the time required for a separate group of the advantages of glucocorticoid.Conclusion:The above 64 cases of severe pemphigus patients in the analysis of the following conclusions can be drawn:1.About 1/3 of the patients were misdiagnosed,suggesting that the necessary for clinical diagnosis.2.Incidence crowd are mainly peasants,workers.3.Hans dominated,accounts for 92.19%.4.Efficacy in the treatment group was significantly higher than that of a separate application of glucocorticoid group.