Background /Objectives

To identify and better understand the clinical features of chronic bullous dermatosis of childhood (CBDC).

Methods

Eligible studies included publication since 1980, CBDC diagnosis, case studies, subjects less than 18 years of age, clinical features and no language restriction. A database search was conducted including Embase, Medline and Scopus on 14th July 2021. See search terms in the appendix. Data was assessed for risk of bias. Jamovi was used statistical analysis. Age and gender were compared to mucocutaneous involvement, cutaneous involvement, other symptoms, human leukocyte antigen (HLA) type and lesion descriptions. Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines (PRISMA) were used.

Results

After duplicate removal using Endnote, of the 351 papers found, 91 met the inclusion criteria. The 130 cases of CBDC include 110 children and 20 neonates. The ratio of male to female neonates is 19:1 and for children is 74:55. Neonates are associated with facial (χ²(1)=9.67,p=0.002), mouth (χ²(1)=31.0,p<0.001), upper airway (χ²(1)=52.7,p<0.001), oesophageal (χ²(1)=34.6,p<0.001), ophthalmic (χ²(1)=6.27,p=0.012) involvement and respiratory distress (χ²(1)=22.7 p<0.001). Children are associated with genital (χ²(1)=3.96,p=0.047), upper (χ²(1)=6.99,p=0.008) and lower limb (χ²(1)=7.03,p=0.008) involvement. Males are associated with facial (χ²(1)=7.01,p=0.008), scalp (χ²(1)=5.96 p=0.015) and perianal (χ²(1)=7.22,p=0.007) involvement.

Conclusion

Limitations include selection bias. The small neonate sample size is unable to be representative of the population. The review highlights the need for further research into the clinical features of CBDC in neonates.