Skip to main content
SpringerLink
Log in

Epidermolysis bullosa acquisita

  • Chapter
Management of Blistering Diseases

Abstract

At the turn of the century, patients were recognized with a blistering disorder that resembled hereditary epidermolysis bullosa, but differed by an adult onset and an absence of a family history of epidermolysis bullosa [1–3]. The term, acquired epidermolysis bullosa (EBA), was coined to categorize this group [1,3]. In order to tighten the diagnosis, Roenigk et al. [4], in 1971 proposed the following clinical criteria for the disease: (1) trauma-induced bullae over the hands, feet, elbows and knees, atrophic scars, milia and nail dystrophy; (2) adult onset; (3) absence of a family history of epidermolysis bullosa; and (4) exclusion of all other bullous diseases. From its inception, acquired epidermolysis bullosa has represented an operational categorization. Through the years, concepts concerning these patients have gradually evolved. Specific diseases that might have been included in EBA as defined above were removed following their recognition.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 39.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 54.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Elliott, G.T. (1895) Two cases of epidermolysis bullosa. J. Cutan. Genitourin. Dis., 13, 8–10.

    Google Scholar 

  2. Wise, F. and Lautman, M.F. (1915) Epidermolysis bullosa beginning in adult life. The acquired form of the disease, with the report of a case and review of the literature. J. Cutan. Dis., 33, 44–52.

    Google Scholar 

  3. Kablitz, R. (1904) Ein Reitrag Zur Frage der Epidermolysis Bullosa (hereditaria et acquisita) Rostock. (Dissertation).

    Google Scholar 

  4. Roenigk, H.H. Jr., Ryan, J.G. and Bergfeld, W.F. (1971) Epidermolysis bullosa acquisita: report of three cases and review of all published cases. Arch. Dermatol., 103, 1–10.

    Article  PubMed  Google Scholar 

  5. Müller, S.A., Sams, W.M. and Dobson, R.L. (1969) Amyloidosis masquerading as epidermolysis bullosa acquisita. Arch. Dermatol., 99, 739–47.

    Article  PubMed  Google Scholar 

  6. Beer, W.E. and Cooke, K.B. (1967) Epidermolysis bullosa induced by penicillamine. Brit. J. Dermatol., 79, 123–5.

    Article  CAS  Google Scholar 

  7. Harber, L.C. and Bickers, D.R. (1984) Porphyria and pseudoporphyria. J. Invest. Dermatol., 82, 207–9.

    Article  PubMed  CAS  Google Scholar 

  8. Gilchrest, B., Rowe, J. and Mihm, M.C. Jr. (1975) Bullous dermatosis of hemodialysis. Ann. Intern. Med., 83, 480–3.

    Article  PubMed  CAS  Google Scholar 

  9. Kushniruk, W. (1973) The immunopathology of epidermolysis bullosa acquisita. Can. Med. Assoc. J., 108, 1143–6.

    PubMed  CAS  Google Scholar 

  10. Gibbs, R.B. and Minus, H.R. (1975) Epidermolysis bullosa acquisita with electron microscopical studies. Arch. Dermatol., 111, 215–20.

    Article  PubMed  CAS  Google Scholar 

  11. Goodwin, P. and Eady, R. (1977) A case of? epidermolysis bullosa. Clin. Exp. Dermatol., 2, 409–12.

    Article  PubMed  CAS  Google Scholar 

  12. Krivo, J.M. and Miller, F. (1978) Immunopathology of epidermolysis bullosa acquisita: association with mixed cryoglobulinemia. Arch. Dermatol., 114, 1218–20.

    Article  PubMed  CAS  Google Scholar 

  13. Benedetto, A.R., Bergfeld, W.F. and Guirguis, M. (1978) Epidermolysis bullosa acquisita diagnosed by immunofluorescence and electronmicroscopy. J. Invest. Dermatol., 70, 221 (abstract).

    Google Scholar 

  14. Livden, J.K., Nilsen, R., Thunold, S. and Schjonsby, H. (1978) Epidermolysis bullosa acquisita and Crohn’s disease. Acta Dermato. venereol. (Stockholm), 58, 241–4.

    CAS  Google Scholar 

  15. Provost, T.T., Maize, J.C., Ahmed, A.R., Strauss, J.S. and Dobson, R.L. (1979) Unusual subepidermal bullous diseases with immunologic features of bullous pemphigoid. Arch. Dermatol., 115, 156–60.

    Article  PubMed  CAS  Google Scholar 

  16. Richter, B.J. and McNutt, N.S. (1979) The spectrum of epidermolysis bullosa acquisita. Arch. Dermatol., 115, 1325–8.

    Article  PubMed  CAS  Google Scholar 

  17. Nieboer, C., Boorsma, D.M., Woerdeman, M.J. and Kalsbeek, G.L. (1980) Epidermolysis bullosa acquisita: immunofluorescence, electron microscopic and immunoelectron microscopic studies in four patients. Brit. J. Dermatol., 102, 383–92.

    Article  CAS  Google Scholar 

  18. Yaoita, H., Briggaman, R.A., Lawley, T.J., Provost, T.T. and Katz, S.I. (1981) Epidermolysis bullosa acquisita: ultrastructural and immunological studies. J. Invest. Dermatol., 76, 288–92.

    Article  PubMed  CAS  Google Scholar 

  19. Roenigk, H.H. Jr and Pearson, R.W. (1981) Epidermolysis bullosa acquisita. Arch. Dermatol., 117, 383.

    Article  PubMed  Google Scholar 

  20. Gammon, W.R., Briggaman, R.A. and Wheeler, C.E. Jr (1982) Epidermolysis bullosa acquisita presenting as an inflammatory bullous disease. J. Amer. Acad. Dermatol., 7, 382–7.

    Article  CAS  Google Scholar 

  21. Gammon, W.R., Briggaman, R.A., Woodley, D.T., Heald, P.W. and Wheeler, C.E. Jr (1984) Epidermolysis bullosa acquisita — a pemphigoid-like disease. J. Amer. Acad. Dermatol., 11, 820–32.

    Article  CAS  Google Scholar 

  22. Caputo, R., Berti, E. and Monti, M. (1982) Pemphigoide bullouse a type d’epidermolyse. J. Dermatol. Paris, 114, 27–8.

    Google Scholar 

  23. Briggaman, R.A., Gammon, W.R. and Woodley, D.T. (1985) Epidermolysis bullosa acquisita of the immunopathological type (dermolytic pemphigoid). J. Invest. Dermatol., 85, 79s–84s.

    Article  PubMed  CAS  Google Scholar 

  24. Lacour, J-P., Juhlin, L., El Baze, P. and Ortonne, J-P. (1985) Epidermolysis bullosa acquisita with negative direct immunofluorescence. Arch. Dermatol., 121, 1183–5.

    Article  PubMed  CAS  Google Scholar 

  25. Unis, M.E., Pfau, R.G., Patel, H., Takahashi, Y. and Anhalt, G. (1985) An acquired form of epidermolysis bullosa without immunoreactants. J. Amer. Acad. Dermatol., 13, 377–80.

    Article  CAS  Google Scholar 

  26. Woodley, D.T., Briggaman, R.A. and Gammon, W.R. (1988) Review and update of epidermolysis bullosa acquisita. Semin. Dermatol., 7, 111–22.

    PubMed  CAS  Google Scholar 

  27. Gammon, W.R., Heise, E.R., Burke, W.A. et al. (1988) Increased frequencies of HLA-DR2 in patients with autoantibodies to epidermolysis bullosa acquisita antigen: evidence that autoimmunity to type VII collagen is HLA class II allele associated. J. Invest. Dermatol. (in press).

    Google Scholar 

  28. Rubenstein, R., Esterly, N.B. and Fine, J.D. (1987) Childhood epidermolysis bullosa acquisita. Detection in a 5 year old girl. Arch. Dermatol., 123, 772–6.

    Article  PubMed  CAS  Google Scholar 

  29. Borok, M., Heng, M.C. and Ahmed, A.R. (1986) Epidermolysis bullosa acquisita in an 8 year old girl. Pediatr. Dermatol., 3, 315–22.

    Article  PubMed  CAS  Google Scholar 

  30. Logan, R.A., Bhogal, B., Das, A.K., McKee, P.H. and Black, M.M. (1986) Localization of bullous pemphigoid antibody — an indirect immuno-fluorescence study of 212 cases using a split-skin technique. Brit. J. Dermatol., 115, (Suppl. 30) 29.

    Google Scholar 

  31. Furue, M., Iwata, M., Yoon, H.I. et al. (1986) Epidermolysis bullosa acquisita: clinical response to plasma exchange therapy and circulating anti-basement membrane zone antibody titer. J. Amer. Acad. Dermatol., 14, 873–8.

    Article  CAS  Google Scholar 

  32. Palestine, R.E., Kossard, S. and Dicken, C.H. (1981) Epidermolysis bullosa acquisita: a heterogenous disease. J. Amer. Acad. Dermatol., 5, 43–53.

    Article  CAS  Google Scholar 

  33. Dahl, M.G.C. (1979) Epidermolysis bullosa acquisita — a sign of cicatricial pemphigoid? Brit. J. Dermatol., 101, 475–83.

    Article  CAS  Google Scholar 

  34. Nilsen, R., Livden, J. and Thunold, S. (1978) Oral lesions of epidermolysis bullosa acquisita. Oral Surg., 45, 749–54.

    Article  PubMed  CAS  Google Scholar 

  35. Lang, P.G. Jr and Tapert, MJ. (1987) Severe ocular involvement in a patient with epidermolysis bullosa acquisita. J. Amer. Acad. Dermatol., 16, 439–43.

    Article  Google Scholar 

  36. Fine, J.D., Tyring, S. and Gammon, W.R. (1988) The presence of intralamina lucida blister formation in epidermolysis bullosa acquisita: possible role of leukocytes. J. Invest. Dermatol. (inpress).

    Google Scholar 

  37. Gammon, W.R., Briggaman, R.A., Inman, A.O. III, Queen, L.L. and Wheeler, C.E. (1984) Differentiating anti-lamina lucida and anti-sublamina densa anti-BMZ antibodies by indirect immunofluorescence on 1.0 M sodium chloride-separated skin. J. Invest. Dermatol., 82, 139–44.

    Article  PubMed  CAS  Google Scholar 

  38. Woodley, D.T., Briggaman, R.A., O’Keefe, E.J. et al. (1984) Identification of the skin basement membrane autoantigen in epidermolysis bullosa acquisita. New Engl. J. Med., 310, 1007–13.

    Article  PubMed  CAS  Google Scholar 

  39. Woodley, D.T., O’Keefe, E.J., Reese, M.J., et al. (1986) Epidermolysis bullosa acquisita antigen, a new major component of cutaneous basement membrane, is a glycoprotein with collagenous domains. J. Invest. Dermatol., 86, 668–72.

    Article  PubMed  CAS  Google Scholar 

  40. Yoshiike, T., Woodley, D.T. and Briggaman, R.A. (1988) Epidermolysis bullosa acquisita: relationship between the collagenase-sensitive and-insensitive domains. J. Invest. Dermatol. (in press).

    Google Scholar 

  41. Palier, A.S., Queen, L.L., Woodley, D.T., et al. (1985) A mouse monoclonal antibody against a newly discovered basement membrane component, the epidermolysis bullosa acquisita antigen. J. Invest. Dermatol., 84, 215–17.

    Article  Google Scholar 

  42. Woodley, D.T., Briggaman, R.A., Gammon, W.R. and O’Keefe, E.J. (1985) Epidermolysis bullosa acquisita antigen is synthesized by human keratinocytes cultured in serum-free medium. Biochem. Biophys. Res. Commun., 130, 1267–72.

    Article  PubMed  CAS  Google Scholar 

  43. Woodley, D.T., Briggaman, R.A., Gammon, W.R., et al. (1986) Epidermolysis bullosa acquista antigen, a major cutaneous basement membrane component is synthesized by human dermal fibroblasts and other cutaneous tissues. J. Invest. Dermatol., 87, 227–31.

    Article  PubMed  CAS  Google Scholar 

  44. Stanley, J.R., Rubinstein, N. and Klaus-Kovtun, V. (1985) Epidermolysis bullosa acquisita antigen is synthesized by both human keratinocyte and human dermal fibroblasts. J. Invest. Dermatol., 85, 542–5.

    Article  PubMed  CAS  Google Scholar 

  45. Lunstrum, G.P., Sakai, L.Y., Keene, D.R., Morris, N.P. and Burgeson, R.E. (1986) Large complex globular domains of type VII procollagen contribute to the structure of anchoring fibrils. J. Biol. Chem., 261, 9042–8.

    PubMed  CAS  Google Scholar 

  46. Sakai, L.Y., Keene, D.R., Morris, N.P. and Burgeson, R.E. (1986) Type VII collagen is a major structural component of anchoring fibrils. J. Cell Biol., 103, 1577–86.

    Article  PubMed  CAS  Google Scholar 

  47. Keene, D.R., Sakai, L.Y., Lunstrum, G.P., Morris, N.P. and Burgeson, R.E. (1987) Type VII collagen forms an extended network of anchoring fibrils. J. Cell Biol., 104, 611–21.

    Article  PubMed  CAS  Google Scholar 

  48. Woodley, D.T., Burgeson, R.E., Lunstrum, G., et al. (1988) The epidermolysis bullosa acquisita antigen is the globular carboxyl terminus of type VII procollagen. J. Clin. Invest., 81, 683–7.

    Article  PubMed  CAS  Google Scholar 

  49. Livden, J.K., Nilsen, R., Thunold, S. and Schjonsby, H. (1978) Epidermolysis bullosa acquisita and Crohn’s disease. Acta Dermatovenereol. (Stockholm), 58, 241–4.

    CAS  Google Scholar 

  50. Ray, T.L., Levine, J.B., Weiss, W. and Ward, P.A. (1982) Epidermolysis bullosa acquisita and inflammatory bowel disease. J. Amer. Acad. Dermatol., 6, 242–52.

    Article  CAS  Google Scholar 

  51. Raab, B., Fretzin, D., Bronson, D., et al. (1983) Epidermolysis bullosa acquisita and inflammatory bowel disease. J. Amer. Med. Assoc, 25, 1746–8.

    Article  Google Scholar 

  52. Burke, W.A., Briggaman, R.A. and Gammon, W.R. (1986) Epidermolysis bullosa acquisita in a patient with multiple endocrinopathies syndrome. Arch. Dermatol., 122, 187–9.

    Article  PubMed  CAS  Google Scholar 

  53. Olansky, A.J., Briggaman, R.A., Gammon, W.R. et al. (1982) Bullous systemic lupus erythematosus. J. Amer. Acad. Dermatol., 7, 511–20.

    Article  CAS  Google Scholar 

  54. Tani, M., Shimizu, R., Ban, M., et al. (1984) Systemic lupus erythematosus with vesiculobullous lesions. Immunoelectron microscopic studies. Arch. Dermatol., 120, 1497–501.

    Article  PubMed  CAS  Google Scholar 

  55. Gammon, W.R., Woodley, D.T., Dole, K.C. and Briggaman, R.A. (1985) Evidence that anti-basement membrane zone antibodies in bullous eruption of systemic lupus erythematosus recognize epidermolysis bullosa acquisita autoantigen. J. Invest. Dermatol., 84, 472–6.

    Article  PubMed  CAS  Google Scholar 

  56. Barton, D.D., Fine, J.D., Gammon, W.R. and Sams, W.M., Jr (1986) Bullous systemic lupus erythematosus: An unusual clinical course and detectable circulating autoantibodies to the epidermolysis bullosa acquisita antigen. J. Amer. Acad. Dermatol., 15, 369–73.

    Article  CAS  Google Scholar 

  57. Hall, R.P., Lawley, T.J., Smith, H.R. and Katz, S.I. (1982) Bullous eruption of systemic lupus erythematosus. Dramatic response to dapsone therapy. Ann. Int. Med., 97, 165–70.

    Article  PubMed  CAS  Google Scholar 

  58. Dotson, A.D., Raimer, S.S., Pursley, T.V. and Tschen, J. (1981) Systemic lupus erythematosus occurring in a patient with epidermolysis bullosa acquisita. Arch. Dermatol., 117, 422–6.

    Article  PubMed  CAS  Google Scholar 

  59. Gammon, W.R., Inman, A.O. III and Wheeler, C.E. Jr (1984) Differences in complement-dependent chemotactic activity generated by bullous pemphigoid and epidermolysis bullosa acquisita immune complexes: demonstration by leukocytic attachment and organ culture methods. J. Invest. Dermatol., 83, 57–61.

    Article  PubMed  CAS  Google Scholar 

  60. Gammon, W.R. and Briggaman, R.A. (1987) Functional heterogeneity of immune complexes in epidermolysis bullosa acquisita. J. Invest. Dermatol., 89, 478–83.

    Article  PubMed  CAS  Google Scholar 

  61. Gammon, W.R., Yancey, K.B. and Hammer, C.H. (1988) Generation of C5 dependent biological activity by basement membrane zone immune complexes. Clin. Res. (in press).

    Google Scholar 

  62. Connolly, S.M. and Sander, H.M. (1987) Treatment of epidermolysis bullosa acquisita with cyclosporine. J. Amer. Acad. Dermatol., 16, 890.

    Article  CAS  Google Scholar 

  63. Zachariae, H. (1987) Cyclosporin A in epidermolysis bullosa acquisita. J. Amer. Acad. Dermatol., 17, 1058–9.

    Article  CAS  Google Scholar 

  64. Crow, L.L., Finkle, J.P., Woodley, D.T. and Gammon, W.R. (1988) Clearing of epidermolysis bullosa acquisita on cyclosporin A. J. Amer. Acad. Dermatol. (in press).

    Google Scholar 

Download references

Authors
  1. Robert A. Briggaman
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. W. Ray Gammon
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. David T. Woodley
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. The Slade Hospital, Oxford, UK

    Fenella Wojnarowska

  2. University of North Carolina School of Medicine, Chapel Hill, USA

    Robert A. Briggaman

Rights and permissions

Reprints and permissions

Copyright information

© 1990 Springer Science+Business Media Dordrecht

About this chapter

Cite this chapter

Briggaman, R.A., Gammon, W.R., Woodley, D.T. (1990). Epidermolysis bullosa acquisita. In: Wojnarowska, F., Briggaman, R.A. (eds) Management of Blistering Diseases. Springer, Boston, MA. https://doi.org/10.1007/978-1-4899-7190-6_10

Download citation

  • DOI: https://doi.org/10.1007/978-1-4899-7190-6_10

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-0-412-28990-3

  • Online ISBN: 978-1-4899-7190-6

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation