A case of subepidermal blistering disease with autoantibodies to multiple laminin subunits who developed later autoantibodies to alpha-5 chain of type IV collagen associated with membranous glomerulonephropathy

Acta Derm Venereol. 2015 Sep;95(7):826-9. doi: 10.2340/00015555-2059.

Abstract

We report a 68-year-old Japanese female patient with subepidermal blistering disease with autoantibodies to multiple laminins, who subsequently developed membranous glomerulonephropathy. At skin disease stage, immunofluorescence demonstrated IgG anti-basement membrane zone antibodies reactive with dermal side of NaCl-split skin. Immunoblotting of human dermal extract, purified laminin-332, hemidesmosome-rich fraction and laminin-521 trimer recombinant protein (RP) detected laminin γ-1 and α-3 and γ-2 subunits of laminin-332. Three years after skin lesions disappeared, nephrotic symptoms developed. Antibodies to α-3 chain of type IV collagen (COL4A3) were negative, thus excluding the diagnosis of Goodpasture syndrome. All anti-laminin antibodies disappeared. Additional IB and ELISA studies of RPs of various COL4 chains revealed reactivity with COL4A5, but not with COL4A6 or COL4A3. Although diagnosis of anti-laminin γ-1 (p200) pemphigoid or anti-laminin-332-type mucous membrane pemphigoid could not be made, this case was similar to previous cases with autoantibodies to COL4A5 and/or COL4A6.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Autoantibodies / analysis*
  • Autoantibodies / blood
  • Autoimmune Diseases / blood
  • Autoimmune Diseases / diagnosis
  • Autoimmune Diseases / immunology*
  • Autoimmune Diseases / therapy
  • Biopsy
  • Blister / blood
  • Blister / diagnosis
  • Blister / immunology*
  • Blister / therapy
  • Collagen Type IV / immunology*
  • Female
  • Fluorescent Antibody Technique
  • Glomerulonephritis, Membranous / blood
  • Glomerulonephritis, Membranous / diagnosis
  • Glomerulonephritis, Membranous / immunology*
  • Glucocorticoids / therapeutic use
  • Humans
  • Kidney / immunology*
  • Kidney / ultrastructure
  • Laminin / immunology*
  • Plasma Exchange
  • Predictive Value of Tests
  • Protein Subunits
  • Skin / drug effects
  • Skin / immunology*
  • Skin / pathology
  • Time Factors

Substances

  • Autoantibodies
  • COL4A5 protein, human
  • Collagen Type IV
  • Glucocorticoids
  • Laminin
  • Protein Subunits