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Childhood bullous pemphigoid associated with IgA antibodies against BP180 or BP230 antigens.
Journal article

Childhood bullous pemphigoid associated with IgA antibodies against BP180 or BP230 antigens.

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  • 1999-04-24
Published in:
  • The British journal of dermatology. - 1999
Antibodies, Anti-Idiotypic
Autoantigens
Child
Child, Preschool
Female
Fluorescent Antibody Technique
Humans
Immunoblotting
Immunoglobulin A
Male
Non-Fibrillar Collagens
Pemphigoid, Bullous
English Linear IgA bullous dermatosis (LABD) comprises a heterogeneous group of subepidermal blistering disorders characterized by in situ bound IgA antibodies in epidermal basement membrane. We report three children presenting clinical and immunopathological features characteristic of LABD. By immunoblotting, the three patients' sera contained IgA antibodies that reacted against the bullous pemphigoid (BP) antigen 180 and or BP230, molecular markers for BP. In addition, IgG antibodies directed against the ectodomain of BP180 were detected by an enzyme-linked immunosorbent assay using a eukaryotic recombinant form of BP180. Consistent with recent studies suggesting that the LABD antigen 1, the predominant autoantigen of LABD, is either a proteolytic product of BP180 or an isoform of the BP180 gene, our findings indicate that a subset of children with features of LABD have a distinct form of BP associated with an IgA response.
Language
  • English
Open access status
closed
Identifiers
Persistent URL
https://sonar.ch/global/documents/65090
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