ADVERTISEMENT
Advertise

Content

List volumes - List articles in this issue

Clinical Report

Epidermolysis Bullosa Acquisita: A Retrospective Clinical Analysis of 30 Cases

doi: 10.2340/00015555-1065

Abstract:

Epidermolysis bullosa acquisita (EBA) is an acquired, autoimmune blistering disorder caused by autoantibody production against type VII collagen. The aim of this study was to examine the clinical types, treatments, and outcomes of 30 patients with EBA. In our cohort, the median age of onset was 44.0 years, with a similar incidence for both genders (46.7% male, 53.3% female). The majority of patients had classic type (36.7%) and bullous pemphigoid (BP)-like type (46.7%) EBA. The remaining patients had mucous membrane pemphigoid-like (6.7%), Brunsting-Perry pemphigoid-like (6.7%), and linear IgA bullous dermatosis-like type (3.3%) EBA. All patients were treated initially with a combination of methylprednisolone, dapsone and colchicine. No significant differences in time to remission were identified between patients with classic vs. BP-like EBA. In a second subset analysis of 19 patients, a group treated with high-dose (> 8 mg) methylprednisolone achieved remission earlier (median time to remission: 3 months) than a group treated with low-dose (≤ 8 mg) methylprednisolone (median time to remission: 12 months), irrespective of clinical type (p = 0.003).

Authors:

Jong Hoon Kim, Yeon Hee Kim, Soo-Chan Kim

References

  • Woodley DT, Briggaman RA, O’Keefe EJ, Inman AO, Queen LL, Gammon WR. Identification of the skin basement-membrane autoantigen in epidermolysis bullosa acquisita. N Engl J Med 1984; 310: 1007–1013.
  • Woodley DT, Burgeson RE, Lunstrum G, Bruckner- Tuderman L, Reese MJ, Briggaman RA. Epidermolysis bullosa acquisita antigen is the globular carboxyl terminus of type VII procollagen. J Clin Invest 1988; 81: 683–687.
  • Woodley DT, Chang C, Saadat P, Ram R, Liu Z, Chen M. Evidence that anti-type VII collagen antibodies are pathogenic and responsible for the clinical, histological, and immunological features of epidermolysis bullosa acquisita. J Invest Dermatol 2005; 124: 958–964.
  • Roenigk HH Jr., Ryan JG, Bergfeld WF. Epidermolysis bullosa acquisita. Report of three cases and review of all published cases. Arch Dermatol 1971; 103: 1–10.
  • Gammon WR, Briggaman RA, Woodley DT, Heald PW, Wheeler CE Jr. Epidermolysis bullosa acquisita – a pemphigoid-like disease. J Am Acad Dermatol 1984; 11: 820–832.
  • Dahl MG. Epidermolysis bullosa acquisita – a sign of cicatricial pemphigoid? Br J Dermatol 1979; 101: 475–484.
  • Kurzhals G, Stolz W, Meurer M, Kunze J, Braun-Falco O, Krieg T. Acquired epidermolysis bullosa with the clinical feature of Brunsting-Perry cicatricial bullous pemphigoid. Arch Dermatol 1991; 127: 391–395.
  • Hashimoto T, Ishiko A, Shimizu H, Tanaka T, Dodd HJ, Bhogal BS, et al. A case of linear IgA bullous dermatosis with IgA anti-type VII collagen autoantibodies. Br J Dermatol 1996; 134: 336–339.
  • Remington J, Chen M, Burnett J, Woodley DT. Autoimmunity to type VII collagen: epidermolysis bullosa acquisita. Curr Dir Autoimmun 2008; 10: 195–205.
  • Hughes AP, Callen JP. Epidermolysis bullosa acquisita responsive to dapsone therapy. J Cutan Med Surg 2001; 5: 397–399.
  • Cunningham BB, Kirchmann TT, Woodley D. Colchicine for epidermolysis bullosa acquisita. J Am Acad Dermatol 1996; 34: 781–784.
  • Miller JL, Stricklin GP, Fine JD, King LE, Arzubiaga MC, Ellis DL. Remission of severe epidermolysis bullosa acquisita induced by extracorporeal photochemotherapy. Br J Dermatol 1995; 133: 467–471.
  • Meier F, Sonnichsen K, Schaumburg-Lever G, Dopfer R, Rassner G. Epidermolysis bullosa acquisita: efficacy of high-dose intravenous immunoglobulins. J Am Acad Dermatol 1993; 29: 334–337.
  • Delgado L, Aoki V, Santi C, Gabbi T, Sotto M, Maruta C. Clinical and immunopathological evaluation of epidermolysis bullosa acquisita. Clin Exp Dermatol 2010; 36: 12–18
  • Gammon WR, Heise ER, Burke WA, Fine JD, Woodley DT, Briggaman RA. Increased frequency of HLA-DR2 in patients with autoantibodies to epidermolysis bullosa acquisita antigen: evidence that the expression of autoimmunity to type VII collagen is HLA class II allele associated. J Invest Dermatol 1988; 91: 228–232.
  • Bernard P, Vaillant L, Labeille B, Bedane C, Arbeille B, Denoeux JP, et al. Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group. Arch Dermatol 1995; 131: 48–52.
  • Zillikens D, Wever S, Roth A, Weidenthaler-Barth B, Hashimoto T, Brocker EB. Incidence of autoimmune subepidermal blistering dermatoses in a region of central Germany. Arch Dermatol 1995; 131: 957–958.
  • Murrell DF, Dick S, Ahmed AR, Amagai M, Barnadas MA, Borradori L, et al. Consensus statement on definitions of disease, end points, and therapeutic response for pemphigus. J Am Acad Dermatol 2008; 58: 1043–1046.
  • Engineer L, Ahmed AR. Emerging treatment for epidermolysis bullosa acquisita. J Am Acad Dermatol 2001; 44: 818–828.
  • Hallel-Halevy D, Nadelman C, Chen M, Woodley DT. Epidermolysis bullosa acquisita: update and review. Clin Dermatol 2001; 19: 712–718.
  • Chen M, O’Toole EA, Sanghavi J, Mahmud N, Kelleher D, Weir D, et al. The epidermolysis bullosa acquisita antigen (type VII collagen) is present in human colon and patients with crohn’s disease have autoantibodies to type VII collagen. J Invest Dermatol 2002; 118: 1059–1064.
  • Ray TL, Levine JB, Weiss W, Ward PA. Epidermolysis bullosa acquisita and inflammatory bowel disease. J Am Acad Dermatol 1982; 6: 242–252.
  • Raab B, Fretzin DF, Bronson DM, Scott MJ, Roenigk HH Jr., Medenica M. Epidermolysis bullosa acquisita and inflammatory bowel disease. JAMA 1983; 250: 1746–1748.
  • Kim ES, Kim WH. Inflammatory bowel disease in Korea: epidemiological, genomic, clinical, and therapeutic characteristics. Gut Liver 2010; 4: 1–14.
  • Ishii N, Hamada T, Dainichi T, Karashima T, Nakama T, Yasumoto S, et al. Epidermolysis bullosa acquisita: what’s new? J Dermatol 2010; 37: 220–230.
  • Hughes BR, Horne J. Epidermolysis bullosa acquisita and total ulcerative colitis. J R Soc Med 1988; 81: 473–475.
  • Related articles

    There are no related articles.


    Share with your friends





    Actions


    Abstract

    Full text

    PDF

    Supplementary


    There is no supplementary for this article.

    Print information


    Volume 91, Issue 3

    DOI: 10.2340/00015555-1065

    Pages: 307-312

    View at PubMed