Clinical features of chronic bullous dermatosis of childhood

Background: Chronic bullous dermatosis of childhood (CBDC) is a rare autoimmune subepidermal blistering disease, which can develop following vaccination or medication, or with an autoimmune condition or illness, among other causes.

Aim: To identify and better understand the clinical features of CBDC by performing a systematic review, in line with the Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines.

Methods: Eligible studies included publication since 1980, CBDC diagnosis, case studies, subjects aged < 18 years, clinical features and no language restriction. A database search was conducted including Embase, MEDLINE and Scopus on 14 July 2021 (see Appendix for search terms). Data were assessed for risk of bias. Jamovi was used for statistical analysis. Age and sex were compared with mucocutaneous involvement, cutaneous involvement, other symptoms, human leucocyte antigen type and lesion descriptions.

Results: After removing duplicate references using Endnote, 351 papers were identified, of which 91 met the inclusion criteria. These papers included 130 cases of CBDC: 110 children and 20 neonates. The ratio of male : female patients was 19 : 1 for neonates and 74:55 for children. χ² analysis with 1 degree of freedom showed that CBDC in neonates was associated with facial (χ²₍₁₎ = 9.67, P < 0.01), 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 = 0.01) involvement and with respiratory distress (χ²₍₁₎ = 22.7 P < 0.001). CBDC in children was associated with genital (χ²₍₁₎ = 3.96, P < 0.05), arm (χ²₍₁₎ = 6.99, P < 0.01) and leg (χ²₍₁₎ = 7.03, P < 0.01) involvement. CBDC in male patients was associated with facial (χ²₍₁₎ = 7.01, P < 0.01), scalp (χ²₍₁₎ = 5.96, P < 0.02) and perianal (χ²₍₁₎ = 7.22, P < 0.01) involvement.

Conclusion: Neonates with CBDC are more likely to have a mucocutaneous distribution of lesions, whereas children are more likely to have cutaneous lesions. The limitations of this study include selection bias, and the small neonate sample size makes the study unrepresentative of the population. The review highlights the need for further research into the clinical features of CBDC in neonates.