A Case of Subepidermal blistering disease with autoantibodies to multiple laminin subunits who developed later autoantibodies to alpha-5 chain of type IV collagen …

H Sueki, Y Sato, S Ohtoshi… - Acta Dermato …, 2015 - medicaljournalssweden.se
H Sueki, Y Sato, S Ohtoshi, T Nakada, A Yoshimura, C Tateishi, DB Borza, W Fader…
Acta Dermato-Venereologica, 2015medicaljournalssweden.se
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 …
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.
medicaljournalssweden.se
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