Infantile bullous pemphigoid

Bullous pemphigoid (BP) is relatively rare in the paediatric age group, with slightly more than 100 cases of BP reported in the literature. In this article, we focus on infantile BP (>= 28 days, <12 months), presenting a systematic review of cases of infantile BP in the literature from 1977 up to the present day. Fifty-four articles were selected, regarding 74 patients with infantile BP. All cases presented involvement of the hands and feet, of which 67/74 (91%) showed subsequent generalized involvement and 7/74 (9%) remained localized. Oral mucosal involvement was reported in 9/62 (15%) patients in whom mucous membranes were investigated. Based on ELISA, all patients had antibodies directed against BPAG2 and only a minority also had antibodies against BPAG1 (8%). Mid-to-high-potency topical steroids BID alone should be used only in mild/localized cases with BSA < 10%. Children with generalized disease with BSA> 10% require systemic steroids, mostly prednisolone at a dosage of 1-2 mg/kg/die. Our study yielded a smaller number of cases of infantile BP, compared to previous reviews, due to the fact that diagnosis of BP was often only based on direct immunofluorescence findings and clinical appearance, without further identification of the targeted auto-antigen. Nevertheless, our findings are in accordance with those of the literature and suggest that BP in infants exhibits specific, characteristic epidemiology, clinical presentation and prognosis.