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.

Sueki H, Sato Y, Ohtoshi S, Nakada T, Yoshimura A, Tateishi C, Borza DB, Fader W, Ghohestani RF, Hirako Y, Koga H, Ishii N, Tsuchisaka A, Qian H, Li X, Hashimoto T
Acta Derm Venereol. 2015 95 (7): 826-9

PMID: 25633161 · DOI:10.2340/00015555-2059

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.

MeSH Terms (18)

Aged Autoantibodies Autoimmune Diseases Biopsy Blister Collagen Type IV Female Fluorescent Antibody Technique Glomerulonephritis, Membranous Glucocorticoids Humans Kidney Laminin Plasma Exchange Predictive Value of Tests Protein Subunits Skin Time Factors

Connections (2)

This publication is referenced by other Labnodes entities:

Links