| BackgroundPemphigus is a relatively rare chronic,recurrent and fatal autoimmune intraepidermal bullous disease,pemphigus is derired from the Greek word"pemphix".Its clinical features are recurrent flaccid blisters and bullae on erythema,normal skin or mucosa,the blisters rupture easily,leaving erosions.Nikolsky's sign is often positive.Histopathological features of the disease are acantholysis,fissures and blisters.Histopathology of different clinical types is different in details.Direct immunofluorescence revealed intracellular anti-IgG and anti-C3 antibodies deposit with reticular pattern,sometimes IgA and IgM antibodies deposit.Indirect immunofluorescence showed pemphigus antibody positive,mainly IgG,sometimes IgA and IgM.The response to treatment is different with different type and severity of pemphigus.The right evaluation of the above contents can guide clinicians to judge the condition and choose the proper treatment.ObjectivesTo summarize the clinical manifestations,pathogenesis,immunological features and treatment of 44 cases of pemphigus with relatively complete data,to provide a basic date for clinicians to better diagnose and treat pemphigus,to improve the prognosis and quality of life of patients.Data and methodsThe clinical data of 44 patients with pemphigus were collected,the patients were hospitalized in Department of Dermatology and Venereology,First Affiliated Hospital of Zhengzhou University from April 2016 to February 2019.All included patients met the clinical and pathological diagnostic criteria for pemphigus.The data of age,gender,clinical manifestation,histopathology,direct immunofluorescence,laboratory examination,complications,treatment and prognosis of the patients were collected and analysed.SPSS21.0 statistical software was used for statistical analysis.Mean plus minus standard deviation was used to desiribe the measurement of data with normal distribution,t-test was carried out to compare the data of different groups;median(25 quartile,75 quartile)were used to desiribe the measurement of data with non-normal distribution,and rank sum test was carried out to compare the data of two independent samples.Spearman correlation analysis was used for correlation analysis.P<0.05 was considered statistically significant.Results1 AgeAmong 44 pemphigus patients,the onset age ranged from 28 to 84 years,with an average age of 56.39±13.66 years.The median duration of the disease was 7.00months,ranging from 0.5 to 156 months at the time of visiting our hospital.2 GenderAmong 44 pemphigus patients,22 were male and 22 were female.The ratio of male to female was 1:1.3 Clinical typesOf the 44 patients with pemphigus,70.45%were pemphigus vulgaris,25.00%were pemphigus foliaceus(8 of them were erythematous pemphigus),2.27%was paraneoplastic pemphigus,and 2.27%was pemphigus herpetiformis.4 Clinical manifestations4.1 Skin lesions:Typical lesions were erythema of skin or mucosa,flaccid bullae and erosions of varying degrees.Among 44 pemphigus patients,77.27%were positive for Nikolsky's sign,9.09%were suspicious positive,and 13.64%were negative.4.2 The first site of skin lesions:Of the 44 patients,43.18%of the patients had skin lesions on the trunk and limbs at onset,18.18%of the patients had skin lesions on the head,and 38.64%of the patients had skin lesions in the mouth.4.3 Skin involvement:Of the 44 patients,95.45%involved the skin,and the ABSIS skin scores were 1 to 80 points with a median score of 25.00.4.4 Mucosal involvement:Of the 44 patients,27 patients involved the mucosal membrane,of which 22 were only affected oral mucosa and 5 were affected oral and vulvar mucosa.The ABSIS mucosa extent scores of 27 patients with oral mucosal lesions were 1 to 11 with a median score of 6.The ABSIS oral mucosal severity scores were 4.5 to 37 with a median score of 27.00.5 Histopathology and Direct immunofluorescenceThe 44 patients showed different degrees of acantholysis.The acantholysis were located above the basal layer in 32 cases and the granular layer or subcorny layer in12 cases.Direct immunofluorescence was performed in 32 cases.Intercellular reticular antibody deposits were found in involved skin.IgG and C3 antibodies deposit were found in 17 cases,IgG antibody deposits in 9 cases and C3 antibody deposits in 6 cases.6 Serum pemphigus antibody levelDsg1 antibody level was increased in 41 patients,with an average of 160.25+75.39 U/ml,Dsg3 antibody level was increased in 29 patients,with an average of180.43±63.81 U/ml.The level of Dsg1 antibody was positively correlated with ABSIS skin score and no correlation with ABSIS mucosal score,while the level of Dsg3 antibody was highly positively correlated with ABSIS mucosal score and no correlation with ABSIS skin score.7 Count of T lymphocyte in peripheral blood7.1 The T lymphocyte(CD3~+)decreased in 23 patients of pemphigus;the Th cells(CD4~+)decreased in 24 patients;the Ts cells(CD8~+)decreased in 10 patients and increased in 1 patient;the ratio of CD4~+/CD8~+decreased in 10 patients and increased in 3 patients.7.2 There was no correlation between CD3~+,CD4~+,CD8~+T lymphocyte count and CD4~+/CD8~+ratio in peripheral blood of pemphigus patients and ABSIS skin score and ABSIS mucosa score.8 Complicated diseasesAmong 44 pemphigus patients,13 were complicated with hypertension,9 with diabetes,5 with skin bacterial infection,4 with coronary heart disease,1 with asthma and chronic obstructive pulmonary disease,1 with thymoma,1 with Castleman disease,1 with interstitial pneumonia,1 with iron deficiency anemia,and 6 with two of the above diseases.9 Misdiagnosis14 out of 44 pemphigus patients had been misdiagnosed,the misdiagnosis rate was 31.82%.10 Treatments10.1 All 44 patients were treated with systemic therapy.One patient received methotrexate alone,one patient received cyclophosphamide alone,13 patients received methylprednisolone alone,and 29 patients received methylprednisolone combined with immunosuppressive treatment,immunosuppressive agents include(CTX,MTX,MMF,CsA).There was no significant difference in the initial dose,maximum dose and daily average dose of steroid between the two treatment regimens(P>0.05).The median hospitalization time of the former was 10 days,and the median hospitalization time of the latter was 17 days.The difference was statistically significant(P<0.01).10.2 Of the 44 patients with pemphigus in this study,6 patients were treated with intravenous immunoglobulin during hospitalization because of their serious condition,and 1 patient with paraneoplastic pemphigus was transferred to departerment of thoracic surgery after discovering the complication of Castleman's disease.10.3 The median hospital stay of 44 patients was 12 days,and all of them were discharged from hospital after their condition improved.Conclusions1.The level of Dsg1 antibody was positively correlated with ABSIS skin score,suggesting that the level of Dsg1 antibody could reflect the severity of skin lesion;while the level of Dsg3 antibody was positively correlated with ABSIS mucosal score,suggesting that the level of Dsg3 antibody could reflect the severity of mucosal lesion.2.Cell-mediated immune function abnormalities were abserved in pemphigus,and may play a role in pathojenesis of pemphigus.3.Pemphigus should be valued according to the clinical manifestation,serum pemphigus antibody level and cell-mediated immune function of the patients,and treated individualized. |
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