Bullous pemphigoid in infancy: Clinical and epidemiologic characteristics

Background: Recent cases of infants with bullous pemphigoid (BP) prompted us to explore the clinical and laboratory features of childhood BP. Objectives: We sought to explore the characteristics of infantile BP and compare them with childhood BP. Methods: All new consecutive cases of infantile BP referred to dermatologic departments in Israel during 2004 to 2006 were retrospectively reviewed. All reported cases in the English- and foreign-language medical literature were gathered and statistical analysis of all cases was performed. Results: Reports on infantile BP are rapidly increasing. Among 78 reported children with BP, 42 (53%) occurred in the first year of life. The incidence of infantile BP in Israel in the last years is 2.36:100,000/y. Predisposition for acral involvement is significantly higher in infantile BP than in childhood BP (79% vs 17%, P < .001), whereas genital involvement is very rare (5% vs 44%, P = .002). Laboratory parameters were not significantly different, except for a more frequent IgM deposition at the dermoepidermal junction in childhood BP (29% vs 10%, P = .042). Limitations: Statistical analyses of published cases may not be representative and could be affected by possible reporting biases. Conclusions: Infantile BP may not be as rare as commonly stated. Age-related differences in regional distribution of lesions in BP were demonstrated. No major differences regarding laboratory results, treatment, and prognosis were found.