Background: Bullous pemphigoid (BP) in infants is a rare
but increasingly reported autoimmune blistering skin
disease. Autoantibody reactivity is usually poorly characterized.
Current guidelines do not address specific aspects of
the infantile form of BP.
Methods: Detailed characterization of a current case series
of five infants with BP from our departments. Comprehensive
analysis of all reported cases (1–12 months) with
respect to clinical and laboratory characteristics, treatment
and outcome.
Results: In total 81 cases were identified. The mean age
was 4.5 months. Moderately severe and severe disease was
seen in 84% of cases. Immunofluorescence microscopy was
comparable with BP in adults. Where analyzed, the NC16A
domain of bullous pemphigoid 180 kDa antigen / collagen
XVII (BP180) was identified as the major target antigen.
BP180 NC16A ELISA values in our cohort were significantly
higher than in a control cohort of 28 newly diagnosed adult
patients.
50% of patients were treated with systemic corticosteroids,
20% with a combination of systemic corticosteroids and
dapsone or sulfapyridine and 10% with topical corticosteroids
alone. 14% of patients needed a combination of
multiple immunosuppressants. All but one patient reached
remission. Relapses were rare.
Conclusions: Presentation of infantile BP is often severe
with blistering of hands and feet present in all cases. Pathogenesis and diagnostic criteria are comparable to adult BP,
yet BP180 NC16A ELISA levels seem to be significantly
higher in infants. The overall disease outcome is favorable.
Based on the results of this study we propose a treatment
algorithm for infantile BP.