Concomitant Occurrence of Circulating IgA Anti‐intercellular and Anti‐basement Membrane Zone Antibodies in Autoimmune Blistering Diseases: Immunofluorescence and Immunoblot Studies

Abstract

Recently, cases with circulating IgA anti-intercellular antibodies have been described. The objective of this study was to present immunofluorescence and immunoblot findings in three cases of bullous diseases with concomitant circulating IgA anti-intercellular and anti-basement membrane zone antibodies. Direct immunofluorescence, indirect immunofluorescence on intact and 1M NaCl-split skin, immunoblotting of epidermal extracts from dispase- and EDTA-separated (two different procedures) human skin, and immunoblotting of the bovine desmosome preparation were performed. All three cases had IgA anti-intercellular and anti-basement membrane zone antibodies. However, immunoblot results were divergent. Case 1 had antibodies against the 150 kD pemphigus foliaceus antigen (IgG), the 170 kD protein (IgG and IgA), and the 97 kD antigen (IgG and IgA). Case 2 had IgG antibodies reactive with the 230 kD and the 170 kD bullous pemphigoid antigens, while case 3 had IgA antibodies against the 97 kD antigen only. The results of immunofluorescence and immunoblot studies in our patients widen the spectrum of laboratory features in blistering skin diseases mediated, at least in part, by antibodies of the IgA class.

Immunomapping of EBA sera to multiple epitopes on collagen VII: further evidence that anchoring fibrils originate and terminate in the lamina densa

“…Dermal protein extracts containing type VII collagen were prepared using EDTA split skin according to the methods described by Dmochowski (20,21). Briefly, normal human foreskin was split by incubation with EDTA in phosphate buffered saline with phenyl methyl sulphonyl fluoride.…”

Molecular Biological Aspects of Acquired Bullous Diseases

“…Immunoelectron microscopy studies as well as studies with salt-split skin indicate that serum from one group of patients binds to antigens in the lamina lucida and serum from another group of patients binds to antigens on the dermal side of the split, indicating a heterogeneous nature of linear IgA bullous dermatosis (Dmochowski et al, 1993;Zone, 1993). The first group of linear IgA bullous dermatosis patients, who had antibodies localized to the lamina lucida, recognized a 97-kDa peptide in human skin extracts by Western blot analysis.…”

Mechanisms of epithelial damage: are there parallels between bullous skin diseases and asthma?

“…Over the last few years evidence has accumulated documenting the incidence of basement membrane zone (BMZ) autoantibodies in blistering skin disorders such as bullous pemphigus [1], cicatricial pemphigoid [2], linear IgA disease of adults (LAD) and chronic bullous disease of childhood (CBDC) [3,4]. Regarding their underlying aetiology and histopathology, these conditions appear to bear some resemblance to asthma.…”

“…Autoantibodies in bullous disorders are detectable in skin biopsies at the site of lesion and in serum by direct and indirect immunofluorescence techniques [4,5]. In biopsies of bullous skin, autoantibodies are localized to the basement membrane and may additionally be localized to the intercellular region of epidermal cells [4].…”

“…Autoantibodies in bullous disorders are detectable in skin biopsies at the site of lesion and in serum by direct and indirect immunofluorescence techniques [4,5]. In biopsies of bullous skin, autoantibodies are localized to the basement membrane and may additionally be localized to the intercellular region of epidermal cells [4]. Allied to the presence of autoantibodies may be the presence of linear deposits of complement factors simi-Correspondence: D. Dhami, Immunopharmacology Group, University Medicine and Clinical Pharmacology, Southampton General Hospital, Southampton SO9 4XY, UK.…”