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.
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Authors | Hirohiko Sueki, Yoshinori Sato, Shinpei Ohtoshi, Tokio Nakada, Ashio Yoshimura, Chiharu Tateishi, Dorin-Bogdan Borza, William Fader, Reza F Ghohestani, Yoshiaki Hirako, Hiroshi Koga, Norito Ishii, Atsunari Tsuchisaka, Hua Qian, Xiaoguang Li, Takashi Hashimoto |
Journal | Acta dermato-venereologica
(Acta Derm Venereol)
Vol. 95
Issue 7
Pg. 826-9
(Sep 2015)
ISSN: 1651-2057 [Electronic] Sweden |
PMID | 25633161
(Publication Type: Case Reports, Journal Article)
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Chemical References |
- Autoantibodies
- COL4A5 protein, human
- Collagen Type IV
- Glucocorticoids
- Laminin
- Protein Subunits
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Topics |
- Aged
- Autoantibodies
(analysis, blood)
- Autoimmune Diseases
(blood, diagnosis, immunology, therapy)
- Biopsy
- Blister
(blood, diagnosis, immunology, therapy)
- Collagen Type IV
(immunology)
- Female
- Fluorescent Antibody Technique
- Glomerulonephritis, Membranous
(blood, diagnosis, immunology)
- Glucocorticoids
(therapeutic use)
- Humans
- Kidney
(immunology, ultrastructure)
- Laminin
(immunology)
- Plasma Exchange
- Predictive Value of Tests
- Protein Subunits
- Skin
(drug effects, immunology, pathology)
- Time Factors
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