Antiepiligrin Cicatricial Pemphigoid

Leverkus, Martin; Schmidt, Enno; Lazarova, Zelmira; Bröcker, Eva B.; Yancey, Kim B.; Zillikens, Detlef

doi:10.1001/archderm.135.9.1091

Cited by 70 publications

(15 citation statements)

References 34 publications

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“…The marked laryngeal involvement in the cases suggests that autoantibodies to lam332 may be responsible. This correlates with a report of 4 out of 5 lam332‐positive MMP patients having laryngeal involvement 5 . Two out of six patients in Oxford with lam332 MMP have marked laryngeal involvement.…”

Section: Comparison Of the Features Of Mmp And H‐jeb And Nhjebsupporting

confidence: 91%

“…The laryngeal complications occur in less than 10% of all MMP cases, but at least 80% of laminin 5 (lam332)–associated MMP cases 5 (particularly the G domain of the alpha 3 chain 6 ), which is the antigen in 5% of MMP cases. This domain is essential for granulation tissue 7 .…”

Section: Comparison Of the Features Of Mmp And H‐jeb And Nhjebmentioning

confidence: 99%

“…This correlates with a report of 4 out of 5 lam332-positive MMP patients having laryngeal involvement. 5 Two out of six patients in Oxford with lam332…”

mentioning

confidence: 99%

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Mucous membrane pemphigoid: are laminin 5 antibodies a risk factor for laryngeal involvement?

Shipman

Ali

Murrell

et al. 2009

Acad Dermatol Venereol

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Section: Comparison Of the Features Of Mmp And H‐jeb And Nhjebsupporting

confidence: 91%

Section: Comparison Of the Features Of Mmp And H‐jeb And Nhjebmentioning

confidence: 99%

See 1 more Smart Citation

Mucous membrane pemphigoid: are laminin 5 antibodies a risk factor for laryngeal involvement?

Shipman

Ali

Murrell

et al. 2009

Acad Dermatol Venereol

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“…7). About 30% of patients with the clinical phenotype mucous membrane pemphigoid reveal autoantibodies binding to the dermal side of the 1 M NaCl-split skin specific for laminin 5 [97, 98]. The pathogenic relevance of antilaminin 5 antibodies was demonstrated by the passive transfer of antilaminin five antibodies into mice, inducing a sub-epidermal blistering disease [99, 100].…”

Section: Pemphigoid Diseasesmentioning

confidence: 99%

Immunopathology and molecular diagnosis of autoimmune bullous diseases

Mihai

Sitaru

2007

J Cellular Molecular Medi

185

190

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Autoimmune bullous diseases are associated with autoimmunity against structural components maintaining cell–cell and cell matrix adhesion in the skin and mucous membranes. Pemphigus diseases are characterized by autoantibodies against the intercellular junctions and intraepithelial blisters. In pemphigoid diseases and epidermolysis bullosa acquisita, sub-epidermal blistering is associated with autoantibodies targeting proteins of the hemidesmosomal anchoring complex. The autoantigens in autoimmune blistering diseases have been extensively characterized over the past three decades. In general, the pathogenicity of autoantibodies, already suggested by clinical observations, has been conclusively demonstrated experimentally. Detection of tissue-bound and circulating serum autoantibodies and characterization of their molecular specificity is mandatory for the diagnosis of autoimmune blistering diseases. For this purpose, various immunofluorescence methods as well as immunoassays, including immunoblotting, enzyme-linked immunosorbent assay and immunoprecipitation have been developed. This review article describes the immunopathological features of autoimmune bullous diseases and the immunological and molecular tests used for their diagnosis and monitoring.

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“…Laminin 5 consists of laminin α3, β3 and γ2 chains and is located in lamina densa under the hemidesmo‐some structure of epidermal keratinocytes (18,19). The majority of AECP patients have autoantibodies to the α3 subunit of the protein (5), but some patients have antibodies to laminin β3, and others show reactivity to the α3 and γ2 subunits or to the β3 and γ2 subunits (4, 5). The present case had antibodies to the laminin β3 and γ2 subunits.…”

Section: Discussionmentioning

confidence: 99%

A Case of Antiepiligrin Cicatricial Pemphigoid with Nephrotic Syndrome

Uchino

Kanekura

Takeda

et al. 2004

The Journal of Dermatology

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We report a 71-year-old woman with antiepiligrin cicatricial pemphigoid associated with nephrotic syndrome. She presented with a six-month history of pruritic blisters over her trunk and legs. She also had episodes of recurrent painful oral erosions. A skin biopsy showed a subepidermal bulla, and a direct immunofluorescence (DIF) study revealed linear deposition of IgG and C3 at the basement membrane zone (BMZ). Indirect immunofluorescence (IIF) staining of 1 M NaCl-split skin demonstrated circulating IgG autoantibodies reactive with the dermal side. Immunoprecipitation studies of the patient's serum disclosed IgG autoantibodies directed against a set of polypeptides that corresponded to laminin 5 (beta 3 gamma 2). Based upon the long-standing edema of her legs and her hypoproteinemia, she was diagnosed with nephrotic syndrome. To our knowledge, the association of antiepiligrin cicatricial pemphigoid with nephrotic syndrome has not been reported previously.

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Antiepiligrin Cicatricial Pemphigoid

Cited by 70 publications

References 34 publications

Mucous membrane pemphigoid: are laminin 5 antibodies a risk factor for laryngeal involvement?

Mucous membrane pemphigoid: are laminin 5 antibodies a risk factor for laryngeal involvement?

Immunopathology and molecular diagnosis of autoimmune bullous diseases

A Case of Antiepiligrin Cicatricial Pemphigoid with Nephrotic Syndrome

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