title: Bullous Pemphigoid With a Dual Pattern of Glomerular Immune Complex Disease.
abstract: A 75-year-old man presented with a blistering skin disease and nephrotic syndrome. Bullous pemphigoid was diagnosed by linear immunoglobulin G (IgG) and C3 staining along the basement membrane zone of a skin biopsy specimen and by the presence of circulating IgG recognizing the 180-kDa bullous pemphigoid antigen (BP180; type XVII collagen). A kidney biopsy specimen showed endocapillary inflammation without crescents. Direct immunofluorescence showed strong IgG and C3 staining in a combined granular and linear pattern along the glomerular basement membrane. Electron microscopy showed subepithelial deposits. In serum, no antibodies against the Goodpasture antigen (type IV collagen) or phospholipase A2 receptor were detected. Indirect immunofluorescence studies using the patient's serum showed a strikingly linear but not granular IgG pattern along the epithelial basement membranes of monkey esophagus and kidney. Although type XVII collagen was recently identified in the glomerulus, the patient's serum did not produce a 180-kDa band on immunoblot of kidney tissue and still stained glomeruli of BP180 knockout mice by indirect immunofluorescence. The patient was treated with prednisone and azathioprine, which resulted in complete remission of skin and kidney manifestations. Although bullous pemphigoid has been reported previously in association with anti-glomerular basement membrane disease or membranous nephropathy, this case demonstrates both elements in 1 patient. This concurrence and the linear pattern on indirect immunofluorescence support the possibility of cross-reactive or parallel autoantibodies to basement membranes with a secondary membranous component.
authors.affiliation: Department of Internal Medicine, Erasmus Medical Center, Rotterdam, the Netherlands. Electronic address: e.j.hoorn@erasmusmc.nl.
authors.forename: Ewout J
authors.initials: EJ
authors.lastname: Hoorn
authors.affiliation: Department of Immunology, Erasmus Medical Center, Rotterdam, the Netherlands.
authors.forename: Noor E
authors.initials: NE
authors.lastname: Taams
authors.affiliation: Department of Dermatology, Medical Research Center Oulu, University of Oulu and Oulu University Hospital, Oulu, Finland.
authors.forename: Tiina
authors.initials: T
authors.lastname: Hurskainen
authors.affiliation: Department of Internal Medicine, Erasmus Medical Center, Rotterdam, the Netherlands.
authors.forename: Mahdi
authors.initials: M
authors.lastname: Salih
authors.affiliation: Department of Pathology, Erasmus Medical Center, Rotterdam, the Netherlands.
authors.forename: Jan J
authors.initials: JJ
authors.lastname: Weening
authors.affiliation: Department of Dermatology, University Medical Center Groningen, Groningen, the Netherlands.
authors.forename: Marcel F
authors.initials: MF
authors.lastname: Jonkman
authors.affiliation: Department of Dermatology, University Medical Center Groningen, Groningen, the Netherlands.
authors.forename: Hendri H
authors.initials: HH
authors.lastname: Pas
authors.affiliation: Department of Immunology, Erasmus Medical Center, Rotterdam, the Netherlands.
authors.forename: Marco W J
authors.initials: MW
authors.lastname: Schreurs
mesh_terms: D000368:Aged; D000818:Animals; D005921:Glomerulonephritis / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D007105:Immune Complex Diseases / Q000150:complications / Q000175:diagnosis*; D008297:Male; D051379:Mice; D018345:Mice, Knockout; D010391:Pemphigoid, Bullous / Q000150:complications / Q000175:diagnosis*
keywords: 180-kDa BP antigen; Bullous pemphigoid; anti-GBM disease; autoantibody; glomerular basement membrane (GBM); membranous nephropathy; nephrotic syndrome; renal biopsy; type XVII collagen