Pediatric epidermolysis bullosa acquisita: A review. Issue 5 (2nd August 2021)

• Record Type: Journal Article
• Authors: Hignett, Emma; Sami, Naveed
• Is Part Of: Pediatric dermatology. Volume 38:Issue 5(2021)
• Page Start: 1047
• Page End: 1050
• Publication Date: 2021-08-02
• Subjects: bullous skin disease -- childhood -- epidermolysis bullosa acquisita -- juvenile -- pediatric; Pediatric dermatology -- Periodicals; Children -- Diseases -- Periodicals; 618.925
• DOI: 10.1111/pde.14722
• Languages: English
• ISSNs: 0736-8046

Abstract

Abstract: Epidermolysis bullosa acquisita (EBA) is an acquired autoimmune blistering skin disorder that is rare in adults and even rarer in childhood. This review aims to identify cases of pediatric EBA and report their clinical features and course. Our literature review was conducted in MEDLINE ® using the search terms related to juvenile epidermolysis bullosa acquisita. We identified 40 cases of pediatric EBA. Mucosal tissues were affected in 29 out of 40 cases. Treatment mostly consisted of a systemic corticosteroid combined with dapsone. Prognosis is favorable with 17 of 40 cases achieving complete remission, 9 of 40 with complete control with therapy, 12 of 40 with partial control with therapy, 1 of 40 with no response to therapy, and 1 of 40 terminating treatment early. Though it is a rare condition, childhood EBA should still be included in the differential diagnosis of pediatric blistering diseases.