“… 5 Drug‐induced BP can be acute and self‐limiting, with resolution following withdrawal of the offending drug, or persistent, in which the drug appears to have initiated idiopathic BP, and this was observed in our patient. 6 IMF studies may reveal tissue‐bound and circulating anti‐BMZ antibodies, 4 , 7 and the antigen in drug‐induced BP has been identified as identical to that in idiopathic BP, 6 as in our case.…”
“… 5 Drug‐induced BP can be acute and self‐limiting, with resolution following withdrawal of the offending drug, or persistent, in which the drug appears to have initiated idiopathic BP, and this was observed in our patient. 6 IMF studies may reveal tissue‐bound and circulating anti‐BMZ antibodies, 4 , 7 and the antigen in drug‐induced BP has been identified as identical to that in idiopathic BP, 6 as in our case.…”
“…Its incidence is increasing [ 6 , 7 ] and it mostly affects the elderly; it is considered rare in children [ 8 , 9 ]. The first case of BP in a child was described in 1970 based on immunofluorescence diagnosis [ 10 ]; the first case of BP in an infant was described in 1977 [ 11 ]. Since then, the number of reported pediatric cases has steadily increased, prompting Nemeth et al to propose diagnostic criteria for childhood BP [ 12 ] which included children and adolescents up to 18 years of age.…”
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.
“…Most of the previously reported cases of BP in childhood were treated with prednisolone (30-120 mg daily for up to 4 years) (Fincher, Dupree & Bean, 1971;Robison & Odom, 1978;Chorzelski & Jablonska, 1979) and some were given additional azathioprine or methotrexate (Bean, Good & Windhorst, 1970;Esterly et al, 1973). However, the findings reported here, and those of Piamphongsant, Chaikittisilpa & KuUavanijaya (1977), show that dapsone or sulphapyridine can effectively control the disease.…”
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