BackgroundBullous 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. The objectives of this study are to define clinical and diagnostic characteristics of infantile BP and develop a treatment algorithm.MethodsDetailed 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.ResultsIn total 81 cases were identified (including our own). The mean age was 4.5 months. Moderately severe and severe disease was seen in 84% of cases. Involvement of hands and feet was present in all 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.ConclusionsPresentation 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.Electronic supplementary materialThe online version of this article (doi:10.1186/s13023-014-0185-6) contains supplementary material, which is available to authorized users.

A study into growth and anaemia in children with epidermolysis bullosa Summary Severe subtypes of epidermolysis bullosa (EB) are devastating conditions that cause extremely fragile skin and mucosal membranes and lots of wounds. So far, no cure for EB exists. About 5,000 people in the U.K. and 500,000 people worldwide suffer from different EB types.Apart from skin problems, children with EB often have trouble gaining weight, and they develop anaemia (deficiency of iron). As wound healing works best when the body is strong, researchers needed to see how exactly children with EB grow and what factors may stop them from growing properly. This is a summary of the study: Natural history of growth and anaemia in children with epidermolysis bullosa: a retrospective cohort study This summary relates to https://doi.

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