Archives of Dermatological Research

, Volume 280, Issue 6, pp 333–337 | Cite as

Autoantibodies to extracellular collagen matrix components in epidermolysis bullosa and other bullous diseases

  • S. Gay
  • J. -D. Fine
  • J. S. Storer
Original Contributions


In order to determine whether autoantibodies are present in sera from normal individuals and/or patients with selected bullous disorders, a highly sensitive solid-phase radioimmune assay was established using purified native collagen types I–VI, laminin, and fibronectin as substrates. Sixty-four sera were utilized, representing 12 normal controls as well as 4 patients with extensive thermal burns, 18 with autoimmune bullous diseases (11 bullous pemphigoid, 5 pemphigus vulgaris, and 2 epidermolysis bullosa acquisita), and 30 with non-autoimmune mechanobullous diseases [epidermolysis bullosa (EB): 20 simplex, 4 junctional, and 6 dystrophic]. In general, autoantibodies to types I, II, and VI collagen and fibronectin were undetectable in any of the patient or control groups. In contrast, autoantibodies to types III and V collagen were noted in 87.5% (28/32) and 90.6% (29/32) of EB sera, respectively, while being only rarely noted in sera from other patient groups. Similarly, autoantibodies to type IV collagen and laminin were detected in 50% (16/32) and 40.6% (13/32) of EB sera, especially from patients with simplex and dystrophic forms of the disease. These data suggest that selected interstitial and basement membrane-associated collagens and laminin may become autoimmunogenic in all three forms of inherited EB in contrast to their relative lack of immunogenicity in at least some of the other intraepidermal and subepidermal blistering disorders. The role, if any, of these autoantibodies in the induction or perpetuation of blistering in EB awaits further studies.

Key words

Collagen autoantibodies Epidermolysis bullosa Bullous diseases 


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Copyright information

© Springer-Verlag 1988

Authors and Affiliations

  • S. Gay
    • 1
    • 2
  • J. -D. Fine
    • 1
    • 3
    • 2
  • J. S. Storer
    • 4
  1. 1.Department of Medicine, Institute of Dental ResearchUniversity of Alabama at BirminghamBirminghamUSA
  2. 2.Department of Dermatology, Institute of Dental ResearchUniversity of Alabama at BirminghamBirminghamUSA
  3. 3.Dermatology SectionMedical Service and Birmingham Veterans Administration Medical CenterBirminghamUSA
  4. 4.Department of DermatologyTulane University School of MedicineNew OrleansUSA

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