| Objective:To retrospectively analyze the epidemiology,clinical characteristics,laboratory examinations,infection,treatment and prognosis of patients with pemphigus who were first treated in the Department of Dermatology,First Affiliated Hospital of Dali University from January 2015 to January 2021.At the same time,the risk factors affecting the prognosis of pemphigus patients were explored.To deeply understand the disease characteristics of pemphigus in this region,provide basis for diagnosis and treatment of pemphigus,and improve the survival rate of pemphigus patients.Methods:From January 2015 to January 2021,patients with pemphigus who were hospitalized for the first time in the Department of Dermatology,The First Affiliated Hospital of Dali University and were clinically,histopathologically and immunologically diagnosed were collected.A total of 128 patients who met the criteria were included,their medical records were collected,and relevant data were obtained by telephone follow-up until January2022.The date of diagnosis of pemphigus patients was the starting point of observation,and the end point of observation was the date of death,loss to follow-up or the end of follow-up.Patients lost to follow-up and those who were still alive at the end of followup were regarded as censored data,and the survival time was calculated in months.Statistical analysis of the data was performed with SPSS 26.0 statistical software.Survival analysis was performed using the Kaplan-Meier method to estimate the cumulative mortality rate,and the univariate analysis between groups was performed using the log-rank test（Log-rank test）.<0.05 was considered statistically significant.Graphs were performed using Graphpad Prism 8.3 software.Results:1.Epidemiological data: Among the 128 pemphigus patients in this study,Pemphigus vulgaris（PV）patients accounted for 84.4%,and Pemphigus foliaceus（PF）patients accounted for 12.5%.There were 60 male patients（46.9%）and 68 female patients（53.1%）,with a sex ratio of 1:1.1,and the mean age at onset was 47.0±12.7 years old.81.3% of the pemphigus patients were from rural areas,77.3% of the patients were farmers,and 61.7% of the patients from ethnic minorities.2.Clinical characteristics: Among the pemphigus patients in this study,the first sites of rash included trunk（35.9%）,mucosa（29.7%）,scalp（21.9%）,face（7.0%）,and limbs（5.5%）.34.9% of PV patients had mucosal lesions first,while 63.2% of PF patients had rash first on the trunk.The difference between the two types of pemphigus rash was statistically significant（P<0.05）.During the course of the disease,57.8% of PV patients had mucosal involvement,which was significantly higher than 21.1% of PF patients,with a statistically significant difference（P<0.05）.7.0% of pemphigus patients were negative for Nikolsky sign.The misdiagnosis rate of pemphigus patients before diagnosis is as high as 28.9%.The infection rate of PV patients was 64.2% compared with that of PF patients（36.8%）,and the difference was statistically significant（P < 0.05）.The WBC value of PV patients（11.09（9.12-13.90）*109/L）was higher than that of PF patients（8.51（5.89-12.49）*109/L）,the difference was statistically significant（P < 0.05）.89 strains were detected in the secretion culture of pemphigus patients,including 84.2% G+ strain,14.7% G-strain and only 1.1% fungus.Staphylococcus aureus（46.1%）and Staphylococcus epidermis（16.9%）were the two strains with the highest detection rate.G+ strain was sensitive to furantoin and vancomycin,and the resistance rate to erythromycin,penicillin G and clindamycin was more than 50%.G-strain was sensitive to amicacin,imipenem,meropenem,cefoperazone/sulbactam,minocycline,and the resistance rate to ampicillin and cefazolin was more than 50%.3.Treatment and prognosis: The antibiotic usage rate of PV patients（49.5%）was significantly higher than that of PF patients（21.1%）,and the difference was statistically significant（P<0.05）.In addition,the cumulative hormone dose before the first dose reduction and the cumulative hormone dose during hospitalization in PV patients were higher than those in PF patients,and the difference was statistically significant（P<0.05）.63.6% of pemphigus patients relapsed due to random drug reduction or withdrawal,and the 1-year hospitalization rate for pemphigus recurrence was 22.7%.4.Survival analysis: The 1-year,3-year and 5-year survival rates of 128 patients with pemphigus were 95.1%,92.1% and 89.5%,respectively.In univariate analysis,the factors affecting the prognosis of pemphigus patients were statistically significant（P<0.05）: age,severity,infection,mucosal damage,WBC,CRP,ALB,BUN,hormone dosage,and the number of re-examinations;multivariate analysis Among the factors affecting the prognosis of pemphigus patients with statistical significance（P<0.05）were age,mucosal damage,ALB,BUN.Conclusion:1.Most of the pemphigus patients in Dali and surrounding areas are middle-aged and elderly people,and the sex ratio of male to female is 1:1.1.Patients with pemphigus mainly come from rural areas,and PV is the most common type of pemphigus.2.Some pemphigus patients only have mucosal involvement in the early stage of the disease,which can easily lead to misdiagnosis of the disease.3.The skin lesions of PV patients mostly first appeared on the mucosal sites,and the skin lesions of PF patients mostly first appeared on the trunk.During the course of the disease,PV patients had higher rates of mucosal involvement,systemic infection and wound infection than PF patients.4.PV patients are more difficult to control the disease than PF patients,and the cumulative amount of hormones required to control the disease is larger.5.The pathogens causing skin wound infection in pemphigus patients are mainly Staphylococcus aureus,which are highly resistant to erythromycin,penicillin G and clindamycin,and are sensitive to nitrofurantoin and vancomycin.6.The recurrence of pemphigus patients is mainly caused by the patients’ random reduction and withdrawal of drugs.7.The 1-year,3-year,and 5-year survival rates of pemphigus patients were 95.1%,92.1%,and 89.5%,respectively.The age at diagnosis was ≥60 years old,with mucosal involvement,increased BUN value,and decreased ALB value of pemphigus patients with poor long-term prognosis. |
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