Retrospective Analysis Of 16 Cases Of Autoimmune Blistering Diseases In Childhood

Objective:This article reviews the clinical characteristics,treatment and prognosis of autoimmune blistering diseases hospitalized children in our hospital,hoping to provide relevant clinical evidence.Methods:This retrospective analysis was carried out on the clinical data of all hospitalized patients in our hospital with AIBD from January 2009 to December 2017,consisting of age,sex,clinical characteristics,laboratory and pathological examination,treatment and prognosis.Results:1.In this study,eight(50.00%)were male,eight(50.00%)were female,the ratio was 1:1.The mean age at disease onset for the children in our group was(3.87 ±4.04)years(range 3 months to13 years).2.The types of AIBD in children in our study were LABD(43.75%),BP(37.50%),PNP(12.50%),and EBA(6.25%),among which LABD is the most common.3.Blisters were mainly affected head,neck,trunk and limbs,and pruritus(81.25%)was the most common initial symptom of AIBD in childhood,mucosal lesions occurred in seven cases(43.75%),a common complication was respiratory tract infection(50.00%).4.Blood routine abnormality was the most common(87.50%),followed by serum levels of immunoglobulins dysfunction(50.00%),and dysfunction of liver was rare.5.Fourteen Patients were treatmented with glucocorticoid or combined with IVIG,diaminodiphenylsulfone and sulfasalazine were used in two cases with LABD respectively.Tumor resection in two cases with PNP.The mean days of hospitalization was(12.43 + 7.67),range 5 to 31 days.One case was cured(6.25%),improved in thirteen cases(81.25%),two cases was unheald(12.50%).Conclusions:1.LABD is the most common type of AIBD in children in our study,followed by BP.2.Compared with adults,the distribution of LABD characteristic annular flowers and beaded vesicles is more typical in children.3.It is not clear for the cause of BP in children,and erythra is more likely to involve the extremities than adults.4.Which particularly important for PNP patients to prolong the survival are early diagnosis and complete resection of the tumor.5.At present,glucocorticoid is considered the first-line systemic therapy for AIBD.The dose of glucocorticoid should be individualized.AIBD in children is sensitive to glucocorticoid,which responses to treatment well,and the prognosis is better than adults.