Linear IgA dermatosis with IgA and IgG autoantibodies to the 180 kDa bullous pemphigoid antigen (BP180): evidence for a distinct subtype.
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.
Metz
Brandie J
BJ
Department of Dermatology, Baylor College of Medicine, One Baylor Plaza FB800, Houston, TX 77030, USA.
Ruggeri
Sun Young
SY
Hsu
Sylvia
S
Reed
Jon A
JA
Ghohestani
Ali S
AS
Uitto
Jouni
J
Ghohestani
Reza F
RF
D000328:Adult; D001323:Autoantibodies / Q000097:blood*; D001324:Autoantigens / Q000276:immunology*; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology; D002352:Carrier Proteins; D003598:Cytoskeletal Proteins; D003937:Diagnosis, Differential; D000071821:Dystonin; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology; D007074:Immunoglobulin G / Q000276:immunology; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D010391:Pemphigoid, Bullous / Q000276:immunology*; D000097805:Collagen Type XVII
D001323:Autoantibodies; D001324:Autoantigens; D002352:Carrier Proteins; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII; C486268:DST protein, human