Studies of patients with anti-epiligrin cicatricial pemphigoid

K. B. Yancey, G. Kirtschig, C. Yee, Z. Lazarova

Research output: Contribution to journalArticle

29 Citations (Scopus)

Abstract

We have recently identified patients with a form of cicatricial pemphigoid who have IgG anti-basement membrane autoantibodies directed against epiligrin, a laminin isoform closely related if not identical to laminin 5. These patients' autoantibodies bind the lower lamina lucida of human epidermal basement membrane and immunoprecipitate this laminin isoform from extracts and media of biosynthetically radiolabeled human keratinocytes. Immunoblot studies show that these patients' autoantibodies specifically bind the α subunit of this laminin (i.e., laminin subunit α3). We have found no evidence of these autoantibodies in normal volunteers or patients with other bullous skin diseases (including those with other forms of CP). These studies have identified a group of patients with an acquired, autoimmune, subepidermal bullous disorder who have disease-specific autoantibodies directed against the α subunit of epiligrin/laminin 5. These findings correlate with prior reports showing that a monoclonal antibody directed against this laminin subunit induces detachment of keratinocytes from extracellular matrix in vitro as well as epidermis from human skin in situ. Together, these findings suggest that this laminin mediates attachment of basal keratinocytes to epidermal basement membrane and that autoantibodies directed against it may be pathogenic. Moreover, recent studies showing that subunits of this laminin isoform are mutated in some patients with Herlitz's junctional epidermolysis bullosa indicate that acquired or inherited abnormalities in this adhesion ligand are associated with skin diseases characterized by separation of epidermis from epidermal BM.

Original languageEnglish (US)
Pages (from-to)829-835
Number of pages7
JournalJournal of Dermatology
Volume22
Issue number11
StatePublished - 1995

Fingerprint

Benign Mucous Membrane Pemphigoid
Laminin
Autoantibodies
Keratinocytes
Basement Membrane
Protein Isoforms
Epidermis
Junctional Epidermolysis Bullosa
Vesiculobullous Skin Diseases
kalinin
Skin Diseases
Extracellular Matrix
Healthy Volunteers
Monoclonal Antibodies
Ligands
Skin

Keywords

  • adhesion molecules
  • autoimmunity
  • bullous diseases

ASJC Scopus subject areas

  • Dermatology

Cite this

Yancey, K. B., Kirtschig, G., Yee, C., & Lazarova, Z. (1995). Studies of patients with anti-epiligrin cicatricial pemphigoid. Journal of Dermatology, 22(11), 829-835.

Studies of patients with anti-epiligrin cicatricial pemphigoid. / Yancey, K. B.; Kirtschig, G.; Yee, C.; Lazarova, Z.

In: Journal of Dermatology, Vol. 22, No. 11, 1995, p. 829-835.

Research output: Contribution to journalArticle

Yancey, KB, Kirtschig, G, Yee, C & Lazarova, Z 1995, 'Studies of patients with anti-epiligrin cicatricial pemphigoid', Journal of Dermatology, vol. 22, no. 11, pp. 829-835.
Yancey, K. B. ; Kirtschig, G. ; Yee, C. ; Lazarova, Z. / Studies of patients with anti-epiligrin cicatricial pemphigoid. In: Journal of Dermatology. 1995 ; Vol. 22, No. 11. pp. 829-835.
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