The clinical and immunopathological manifestations of anti-epiligrin cicatricial pemphigoid, a recently defined subepithelial autoimmune blistering disease

C. A. Egan, K. B. Yancey

Research output: Contribution to journalArticle

35 Citations (Scopus)

Abstract

Cicatricial pemphigoid (CP) is a rare, acquired, autoimmune, subepithelial blistering disease. It primarily affects mucous membranes but it also may involve the skin. Morbidity is associated with the propensity for scar formation and may be especially severe when mucosal surfaces such as the conjunctivae, larynx, esophagus, or urethra are involved. On direct immuno-fluorescence microscopy, CP is characterized by the linear deposition of immunoreactants, principally IgG and C3, along epithelial basement memnbranes. Over the last 10 years, studies in a number of laboratories have shown that circulating autoantibodies in patients with CP may target one of several different autoantigens. One subset of patients with the CP-phenotype have IgG anti-basement membrane autoantibodies against laminin 5 (α3β3γ2) (i.e., patients with anti-epiligrin CP [AECP]). This review disncusses recent advances in the understanding of CP and emphasizes salient features of AECP pathophysiology.

Original languageEnglish (US)
Pages (from-to)585-589
Number of pages5
JournalEuropean Journal of Dermatology
Volume10
Issue number8
StatePublished - 2000

Fingerprint

Benign Mucous Membrane Pemphigoid
Autoimmune Diseases
Autoantibodies
Conjunctiva
Autoantigens
Urethra
Larynx
Fluorescence Microscopy
Basement Membrane
Esophagus
Cicatrix
kalinin
Mucous Membrane
Immunoglobulin G
Morbidity
Phenotype
Skin

Keywords

  • Autoimmunity
  • Epidermal basement membrane
  • Extracellular matrix
  • IgG
  • Laminin

ASJC Scopus subject areas

  • Dermatology

Cite this

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abstract = "Cicatricial pemphigoid (CP) is a rare, acquired, autoimmune, subepithelial blistering disease. It primarily affects mucous membranes but it also may involve the skin. Morbidity is associated with the propensity for scar formation and may be especially severe when mucosal surfaces such as the conjunctivae, larynx, esophagus, or urethra are involved. On direct immuno-fluorescence microscopy, CP is characterized by the linear deposition of immunoreactants, principally IgG and C3, along epithelial basement memnbranes. Over the last 10 years, studies in a number of laboratories have shown that circulating autoantibodies in patients with CP may target one of several different autoantigens. One subset of patients with the CP-phenotype have IgG anti-basement membrane autoantibodies against laminin 5 (α3β3γ2) (i.e., patients with anti-epiligrin CP [AECP]). This review disncusses recent advances in the understanding of CP and emphasizes salient features of AECP pathophysiology.",
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N2 - Cicatricial pemphigoid (CP) is a rare, acquired, autoimmune, subepithelial blistering disease. It primarily affects mucous membranes but it also may involve the skin. Morbidity is associated with the propensity for scar formation and may be especially severe when mucosal surfaces such as the conjunctivae, larynx, esophagus, or urethra are involved. On direct immuno-fluorescence microscopy, CP is characterized by the linear deposition of immunoreactants, principally IgG and C3, along epithelial basement memnbranes. Over the last 10 years, studies in a number of laboratories have shown that circulating autoantibodies in patients with CP may target one of several different autoantigens. One subset of patients with the CP-phenotype have IgG anti-basement membrane autoantibodies against laminin 5 (α3β3γ2) (i.e., patients with anti-epiligrin CP [AECP]). This review disncusses recent advances in the understanding of CP and emphasizes salient features of AECP pathophysiology.

AB - Cicatricial pemphigoid (CP) is a rare, acquired, autoimmune, subepithelial blistering disease. It primarily affects mucous membranes but it also may involve the skin. Morbidity is associated with the propensity for scar formation and may be especially severe when mucosal surfaces such as the conjunctivae, larynx, esophagus, or urethra are involved. On direct immuno-fluorescence microscopy, CP is characterized by the linear deposition of immunoreactants, principally IgG and C3, along epithelial basement memnbranes. Over the last 10 years, studies in a number of laboratories have shown that circulating autoantibodies in patients with CP may target one of several different autoantigens. One subset of patients with the CP-phenotype have IgG anti-basement membrane autoantibodies against laminin 5 (α3β3γ2) (i.e., patients with anti-epiligrin CP [AECP]). This review disncusses recent advances in the understanding of CP and emphasizes salient features of AECP pathophysiology.

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KW - Epidermal basement membrane

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