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Case Reports
. 2004 Jun;43(6):443-6.
doi: 10.1111/j.1365-4632.2004.02016.x.

Linear IgA Dermatosis With IgA and IgG Autoantibodies to the 180 kDa Bullous Pemphigoid Antigen (BP180): Evidence for a Distinct Subtype

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Case Reports

Linear IgA Dermatosis With IgA and IgG Autoantibodies to the 180 kDa Bullous Pemphigoid Antigen (BP180): Evidence for a Distinct Subtype

Brandie J Metz et al. Int J Dermatol. .

Abstract

Background: Autoantibodies in linear immunoglobulin A (IgA) disease (LAD) are reported to be of IgA class and directed against a 97-120 kDa epidermal antigen.

Methods: We report a 39-year-old woman with clinical features of LAD and with circulating IgA and IgG autoantibodies to the 180 kDa bullous pemphigoid antigen (BP180).

Results: Histopathology of lesional skin revealed a subepidermal blister with mixed inflammatory cell infiltrate. Direct immunofluorescence of perilesional skin showed linear deposits of IgA along the dermal-epidermal junction. The antigen specificity of the patient's circulating antibodies was determined by Western blotting and enzyme-linked immunoabsorbent assay (ELISA) using various antigen sources, including cultured human keratinocytes, dermal protein lysates, and purified laminin-5, as well as proteins corresponding to BP180, the 230 kDa bullous pemphigoid antigen (BP230), laminin-5 subunits, and collagen IV alpha1-alpha6 chains. IgA and IgG antibodies in the patient's serum were directed against BP180, and no IgA or IgG reactivity was found against the other skin antigens.

Conclusions: These data provide evidence for the presence of a subtype of LAD with dual IgA and IgG autoimmune response to BP180.

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