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Epidermolysis Bullosa Acquisita

  • Chapter
Autoimmune Diseases of the Skin

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Summary

EBA is a clinically heterogeneous acquired, subepidermal bullous disease. In its classical form, it is a mechanobullous disease with skin fragility and trauma-induced blisters that have minimal inflammation and heal with scarring and milia — features that are highly reminiscent of hereditary dystrophic forms of epidermolysis bullosa. A hallmark of EBA are IgG autoantibodies targeted against the type VII collagen within anchoring fibrils. Anchoring fibrils are structures that anchor the epidermis and its underlying BMZ onto the dermis. However, it has also become evident that EBA may present with clinical manifestations reminiscent of bullous pemphigoid (BP), cicatricial pemphigoid (CP), and Brunsting-Perry pemphigoid. The diagnostic criteria for EBA are: (1) spontaneous or trauma-induced blisters resembling hereditary DEB, (2) adult onset, (3) a negative family history for EB, and (4) the exclusion of all other bullous diseases. Due to the pronounced skin fragility caused by the autoantibody-induced paucity of anchoring fibrils, EBA is difficult to treat. The BP-like inflammatory variants of EBA respond well to systemic corticosteroids and immunosuppressive adjuvants while the mechanobullous variant and the MMP-like variants do to a much lesser extent. Novel therapeutic strategies such as immunoadsorption and the anti-CD20 monoclonal antibody, rituximab, had beneficial effects in small case series.

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References

  • Ackerman AB, Chongchitnant N, Sanchez J, Guo Y, Benin B, Reichel M, Randall MB (1997) Histologic diagnosis of inflammatory skin diseases: an algorithmic method based on pattern analysis. 2nd ed. Williams & Wilkins, Baltimore

    Google Scholar 

  • Arpey CJ, Elewski BE, Moritz DK, Gammon WR (1991) Childhood epidermolysis bullosa acquisita: report of three cases and review of literature. J Am Acad Dermatol 24:706–714

    Article  PubMed  CAS  Google Scholar 

  • Bauer JW, Schaeppi H, Metze D, Muss W, Pohla-Gubo G, Hametner R, Ruckhofer J, Grabner G, Hintner H (1999) Ocular involvement in IgA-epidermolysis bullosa acquisita. Br J Dermatol 141:887–892

    Article  PubMed  CAS  Google Scholar 

  • Bernard P, Vaillant L, Labeille B, Bedane C, Arbeille B, Denoeux JP, Lorette G, Bonnetblanc JM, Prost C (1995) Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Arch Dermatol 131:48–52

    Article  PubMed  CAS  Google Scholar 

  • Borradori L, Caldwell JB, Briggaman RA, Burr CE, Gammon WR, James WD, Yancey KB (1995) Passive transfer of autoantibodies from a patient with mutilating epidermolysis bullosa acquisita induces specific alterations in the skin of neonatal mice. Arch Dermatol 131:590–595

    Article  PubMed  CAS  Google Scholar 

  • Briggaman RA, Wheeler CE Jr (1975) The epidermal-dermal junction. J Invest Dermatol 65:71–84

    Article  PubMed  CAS  Google Scholar 

  • Briggaman RA, Schechter NM, Fraki J, Lazarus GS (1980) Degradation of the epidermal-dermal junction by proteolytic enzymes from human skin and human polymorphonuclear leukocytes. J Exp Med 160:1027–1042

    Article  Google Scholar 

  • Bruckner-Tuderman L, Nilssen O, Zimmermann DR, Dours-Zimmermann MT, Kalinke DU, Gedde-Dahl TJr, Winberg JO (1995) Immunohistochemical and mutation analyzes demonstrate that procollagen VII is processed to collagen VII through removal of the NC-2 domain. J Cell Biol 131:551–559

    Article  PubMed  CAS  Google Scholar 

  • Burgeson RE (1993) Type VII collagen, anchoring fibrils, and epidermolysis bullosa. J Invest Dermatol 101:252–255

    Article  PubMed  CAS  Google Scholar 

  • Callot-Mellot C, Bodemer C, Caux F, Bourgault-Villada I, Fraitag S, Goudie G, Heller M, de Prost Y, Prost C (1997) Epidermolysis bullosa aquisita in childhood. Arch of Dermatol 133:1122–1126

    Article  CAS  Google Scholar 

  • Camara A, Becherel PA, Bussel A, Lagrange S, Chosidow O, Joly P, Piette JC, Frances C (1999) Resistant acquired bullous epidermolysis with severe ocular involvement: the success of extra-corporeal photochemotherapy. Annal Dermatol Venereol 126:612–615

    CAS  Google Scholar 

  • Chan LS, Fine JD, Briggaman RA, Woodley DT, Hammerberg C, Drugge RJ, Cooper KD (1993) Identification and partial characterization of a novel 105-kDalton lower lamina lucida autoantigen associated with a novel immune-mediated subepidermal blistering disease. J Invest Dermatol 101:262–267

    Article  PubMed  CAS  Google Scholar 

  • Chan LS, Woodley DT (1996) Pemphigoid: Bullous and cicatricial. In Current Therapy in Allergy, Immunology and Rhematology, 5th ed, edited by LM Lichtenstein, AS Fauci Mosby, St Louis, p 93

    Google Scholar 

  • Chan LS, Vanderlugt CJ, Hashimoto T, Nishikawa T, Zones JJ, Black MM, Wojnarowask F, Stevens SR, Chen M, Failey JA, Miller SD, Gordon KB (1998) Epitope spreading: Lessons from autoimmune skin diseases. J Invest Dermatol 110:103–109

    Article  PubMed  CAS  Google Scholar 

  • Chen M, Marinkovich MP, Veis A, O’Toole EA, Rao CN, Cai XY, Woodley DT (1997a) Interactions of the amino-terminal noncollagenous (NC1) domain of type VII collagen with extracellular matrix components: a potential role in epidermal-dermal adherence in human skin. J Biol Chem 272:14516–14522

    Article  PubMed  CAS  Google Scholar 

  • Chen M, Chan LS, Cai X, O’Toole EA, Sample JC, Woodley DT (1997b) Development of an ELISA for rapid detection of anti-type VII collagen autoantibodies in epidermolysis bullosa acquisita. J Invest Dermatol 108:68–72

    Article  PubMed  CAS  Google Scholar 

  • Chen M, O’Toole EA, Sanghavi J, Mahmud N, Kelleher D, Woodley DT (1997c) Type VII collagen exists in human intestine and serves as an antigenic target in patients with inflammatory bowel disease. J Invest Dermatol 108:542

    Google Scholar 

  • Chen M, Marinkovich MP, Jones JC, O’Toole E, Li YY, Woodley DT (1999) NC1 domain of type VII collagen binds to the beta3 chain of laminin 5 via a unique subdomain within the fibronectin-like repeats. J Invest Dermatol 112:177–183

    Article  PubMed  CAS  Google Scholar 

  • Chen M, Tahk SH, Costa FK, Kasahara N, Woodley DT (2000a) Recombinant expression of fulllength type VII collagen alpha chain: structural and functional characterization and implication for gene therapy. J Invest Dermatol 114:756

    Google Scholar 

  • Chen M, Wang J, Tahk SH and Woodley DT (2000b) Noncollagenous (NC2) domain of type VII collagen mediates the antiparallel-dimer formation of type VII collagen and constitutes a new antigenic epitope for EBA and BSLE autoantibodies. J Invest Dermatol 114:766

    Google Scholar 

  • Cho HJ, Lee IJ, Kim SC (1998) Complement fixing abilities and IgG subclasses of autoantibodies in epidermolysis bullosa acquisita. Yonsei Medical Journal 39:339–344

    PubMed  CAS  Google Scholar 

  • Choi GS, Lee ES, Kim SC, Lee S (1998) Epidermolysis bullosa aquisita localized to the face. J Dermatol 25:19–22

    PubMed  CAS  Google Scholar 

  • Christiano AM, Greenspan DS, Lee S, and Uitto, J (1994) Cloning of human type VII collagen: complete primary sequence of the alpha 1(VII) chain and identification of intragenic polymorphisms. J Biol Chem 269:20256–20262

    PubMed  CAS  Google Scholar 

  • Connolly SM, Sander HM (1987) Treatment of epidermolysis bullosa acquisita with cyclosporin. J Am Acad Dermatol 16:890

    Article  PubMed  CAS  Google Scholar 

  • Crichlow SM, Mortimer NJ, Harman KE (2007) A successful therapeutic trial of rituximab in the treatment of a patient with recalcitrant, high-titre epidermolysis bullosa acquisita. Br J Dermatol 156:194–196

    Article  PubMed  CAS  Google Scholar 

  • Crow LL, Finkle JP, Gammon WR, Woodley DT (1988) Cleaning of epidermolysis bullosa acquisita on cyclosporin A. J Am Acad Dermatol 19:937–942

    Article  PubMed  CAS  Google Scholar 

  • Cunningham BB, Kirchmann TT, Woodley DT (1996) Colchicine for epidermolysis bullosa (EBA). J Am Acad Dermatol 34:781–784

    Article  PubMed  CAS  Google Scholar 

  • Dahl MG (1979) Epidermolysis bullosa acquisita — a sign of cicatricial pemphigoid? Br J Dermatol 101:475–483

    Article  PubMed  CAS  Google Scholar 

  • De Jong MCJM, Bruins S, Heeres K, Jonkman MF, Nieboer C, Boorsma DM, Pas HH, van der Meer JB (1996) Bullous pemphigoid and epidermolysis bullosa acquisita: Differentiation by fluorescence overlay antigen mapping. Arch Dermatol 132:151–157

    Article  PubMed  Google Scholar 

  • Domloge-Hultsch N, Anhalt GJ, Gammon WR, Lazarova Z, Briggaman R, Welch M, Jabs DA, Huff C, Yancey KB (1994) Antiepiligrin cicatricial pemphigoid: A subepithelial bullous disorder. Arch Dermatol 130:1521–1529

    Article  PubMed  CAS  Google Scholar 

  • Edwards S, Wakelin SH, Wojnarowska F, Marsden RA, Kirtschig G, Bhogal B, Black MM (1998) Bullous pemphigoid and epidermolysis bullosa acquisita: presentation, prognosis, and immunopathology in 11 children. Pediatr Dermatol 15:184–190

    Article  PubMed  CAS  Google Scholar 

  • Elliott GT (1895) Two cases of epidermolysis bullosa. J Cutan Genitourin Dis 13:10

    Google Scholar 

  • Epstein JH, Tuffanelli DL, Epstein WL (1973) Cutaneous changes in the porphyrias. A microscopic study. Arch Dermatol 107:689–698

    Article  PubMed  CAS  Google Scholar 

  • Feliciani C, Di Muzio M, Mohammad Pour S, Allegretti T, Amerio P, Toto P, Coscione G, Proietto G, Amerio P (1998) ’suction split’ as a routine method to differentiate epidermolysis bullosa acquisita from bullous pemphigoid. J Eur Acad Dermatol Venereol 10:243–247

    Article  PubMed  CAS  Google Scholar 

  • Fine JD, Tyring S, Gammon WR (1989) The presence of intra-lamina lucida blister formation in epidermolysis bullosa acquisita: Possible role of leukocytes. J Invest Dermatol 92:27

    Article  PubMed  CAS  Google Scholar 

  • Furue M, Iwata M, Yoon H-I, Kubota Y, Ohto H, Kawashima M, Tsuchida T, Oohara K, Tamaki K, Kukita A (1986) Epidermolysis bullosa acquisita: clinical response to plasma exchange therapy and circulating anti-BMZ antibody titer. J Am Acad Dermatol 14:873–878

    Article  PubMed  CAS  Google Scholar 

  • Gammon WR, Briggaman RA, Wheeler CE Jr (1982) Epidermolysis bullosa acquisita presenting as an inflammatory bullous disease. J Am Acad Dermatol 7:382–387

    Article  PubMed  CAS  Google Scholar 

  • Gammon WR, Briggaman RA, Inman AO, Queen LL, Wheeler CE (1984a) Differentiating antilamina lucida and antisublamina densa anti-BMZ antibodies by indirect immunofluorescence on 1.0 M sodium chloride-separated skin. J Invest Dermatol 82:139–144

    Article  PubMed  CAS  Google Scholar 

  • Gammon WR, Briggaman RA, Woodley DT, Heald PW. Wheeler CE Jr (1984b) Epidermolysis bullosa acquisita — a pemphigoid like disease. J Am Acad Dermatol 11:820–832

    Article  PubMed  CAS  Google Scholar 

  • Gammon WR, Woodley DT, Dole KC, Briggaman RA (1985) Evidence that basement membrane zone antibodies in bullous eruption of systemic lupus erythematosus recognize epidermolysis bullosa acquisita autoantigens. J Invest Dermatol 84:472–476

    Article  PubMed  CAS  Google Scholar 

  • Gammon WR (1988a) Epidermolysis bullosa acquisita. Semin Dermatol 7:218–224

    PubMed  CAS  Google Scholar 

  • Gammon WR, Heise ER, Burke WA, Fine JD, Woodley DT, Briggaman RA (1988b) 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 91:228–232

    Article  PubMed  CAS  Google Scholar 

  • Gammon WR, Kowalewski C, Chorzelski TP, Kumar V, Briggaman RA, Beutner EH (1990) Direct immunofluorescence studies of sodium chloride-separated skin in the differential diagnosis of bullous pemphigoid and epidermolysis bullosa acquisita. J Am Acad Dermatol 22:664–670

    Article  PubMed  CAS  Google Scholar 

  • Gammon WR, Briggaman RA (1993) Epidermolysis bullosa acquisita and bullous systemic lupus erythematosus: disease of autoimmunity to type VII collagen. Dermatol Clin 11:535–547

    Article  PubMed  CAS  Google Scholar 

  • Gammon WR, Murrell DF, Jenison MW, Padilla KM, Prisayanh PS, Jones DA, Briggaman RA, Hunt SW III (1993) Autoantibodies to type VII collagen recognize epitopes in a fibronectin-like region of the noncollagenous (NC1) domain. J Invest Dermatol 100:618–622

    Article  PubMed  CAS  Google Scholar 

  • Ghohestani RF, Nicolas JF, Roussele P, Claudy AL (1997) Diagnostic value of indirect immunofluorescence on sodium chloride-split skin in differential diagnosis of subepidermal autoimmune bullous dermatoses. Arch Dermatol 133:1102–1107

    Article  PubMed  CAS  Google Scholar 

  • Gordon KB, Chan LS, Woodley DT (1997) Treatment of refractory epidermolysis bullosa acquisita with extracorporeal photochemotherapy. Br J Dermatol 136:415–420

    Article  PubMed  CAS  Google Scholar 

  • Harman KE, Whittam LR, Wakelin SH, Black MM (1998) Severe, refractory epidermolysis bullosa acquisita complicated by an oesophageal stricture responding to intravenous immune globulin. Br J Dermatol 139:1126–1127

    Article  PubMed  CAS  Google Scholar 

  • Hashimoto T, Ishiko A, Shimizu H, Tanaka T, Dodd HJ, Bhogal BS, Black MM, Nishikawa T (1996) A case of linear IgA bullous dermatosis with IgA anti-type VII collagen autoantibodies. Br J Dermatol 134:336–339

    Article  PubMed  CAS  Google Scholar 

  • Hoang-Xuan T, Robin H, Heller M, Caux F, Prost C (1997) Epidermolysis bullosa acquisita diagnosed by direct immunoelectron microscopy of the conjunctiva. Ophthalmology 104:1414–1420

    PubMed  CAS  Google Scholar 

  • Jappe U, Zillikens D, Bonnekoh B, Gollnick H (2000) Epidermolysis bullosa acquisita with ultraviolet radiation sensitivity. Br J Dermatol 142:517–520

    Article  PubMed  CAS  Google Scholar 

  • Joly P, Ruto F, Thomine E, Delpech A, Balguerie X, Tron F, Lauret P (1993) Brunsting-Perry Cicatricial Bullous Pemphigoid: a clinical variant of localized acquired epidermolysis bullosa? J Am Acad Dermatol 28:89–92

    Article  PubMed  CAS  Google Scholar 

  • Jones DA, Hunt SW III, Prisayanh PS, Briggaman RA, Gammon WR (1995) Immunodominant autoepitopes of type VII collagen are short, paired peptide sequences within the fibronectin type III homology region of the noncollagenous (NC1) domain. J Invest Dermatol 104:231–235

    Article  PubMed  CAS  Google Scholar 

  • Jonkman MF, Schuur J, Dijk F, Heeres K, de Jong MC, van der Meer JB, Yancey KB, Pas HH (2000) Inflammatory variant of epidermolysis bullosa acquisita with IgG autoantibodies against type VII collagen and laminin α3. Arch Dermatol 136:227–231

    Article  PubMed  CAS  Google Scholar 

  • Kazama T, Yamamoto Y, Hashimoto T, Komai A, Ito M (1998) Application of confocal laser scanning microscopy to differential diagnosis of bullous pemphigoid and epidermolysis bullosa acquisita. Br J Dermatol 138:593–601

    Article  PubMed  CAS  Google Scholar 

  • Kofler H, Wambacher-Gasser B, Topar G, Weinlich G, Schuler G, Hintner H, Romani N, Fritsch P (1997) Intravenous immunoglobulin treatment in therapy — resistant epidermolysis bullosa acquisita. J Am Acad Dermatol 36:331–335

    Article  PubMed  CAS  Google Scholar 

  • Kurzhals G, Stolz W, Meurer M, Kunze J, Braun-Falco O, Krieg T (1991) Acquired epidermolysis bullosa with the clinical feature of Brunsting-Perry cicatricial bullous pemphigoid. Arch Dermatol 127:391–395

    Article  PubMed  CAS  Google Scholar 

  • Kushniruk W (1973) The immunopathology of epidermolysis bullosa acquisita. Can Med Assoc J 108:1143–1146

    PubMed  CAS  Google Scholar 

  • Lapiere JC, Woodley DT, Parente MG, Iwasaki T, Wynn KC, Christiano AM, Uitto J (1993) Epitope mapping of type VII collagen: identification of discrete peptide sequences recognized by sera from patients with acquired epidermolysis bullosa. J Clin Invest 92:1831–1839

    Article  PubMed  CAS  Google Scholar 

  • Lapiere JC, Chen JD, Iwasaki T, Hu L, Uitto J, Woodley DT (1994) Type VII collagen specifically binds fibronectin via a unique subdomain within the collagenous triple helix. J Invest Dermatol 103:637–641

    Article  PubMed  CAS  Google Scholar 

  • Lee CW (1998) Prevalences of subacute cutaneous lupus erythematosus and epidermolysis bullosa acquisita among Korean / oriental papulations. Dermatology 197:187

    Article  PubMed  CAS  Google Scholar 

  • Lee CW (2000) Serum IgA autoantibodies in patients with epidermolysis bullosa acquisita: A high frequency of detection. Dermatology 200:83–84

    Article  PubMed  CAS  Google Scholar 

  • Lever WF, Schaumburg-Lever G (1990) Congenital disease (genodermatoses). In Histopathology of the skin, edited by WF Lever, G Schaumburg-Lever. Philadephia, Lippincott, p 78

    Google Scholar 

  • Lohi J, Leivo I, Tani T, Kiviluoto T, Kivilaakso E, Burgeson RE, Virtanen I (1996) Laminins, tenascin and type VII collagen in colorrectal mucosa. Histochem J 28:431–440

    Article  PubMed  CAS  Google Scholar 

  • Mascaro JM Jr, Zillikens D, Giudice GJ, Caux F, Fleming MG, Katz HM, Diaz LA (2000) A subepidermal bullous eruption associated with IgG autoantibodies to a 200 kd dermal antigen: The first case report from the United States. J Am Acad Dermatol 242:309–315

    Article  Google Scholar 

  • Megahed M, Scharfetter-Kochanek K (1994) Epidermolysis bullosa acquisita-successful treatment with colchicine. Arch Dermatol Res 286:35–46

    Article  PubMed  CAS  Google Scholar 

  • Miller JK, Stricklin GP, Fine JD, King LE, Arzubiaga MC, Ellis DL (1995) Remission of svere epidermolysis bullosa acquisita induced by extracoporeal photochemotherapy. Br J Dermatol 133:467–471

    Article  PubMed  CAS  Google Scholar 

  • Niedermeier A, Eming R, Pfütze M, Neumann CR, Happel C, Reich K, Hertl M (2007) Clinical response of severe mechanobullous epidermolysis bullosa acquisita to combined treatment with immunoadsorption and rituximab (anti-CD20 monoclonal antibodies). Arch Dermatol 143:192–198

    Article  PubMed  CAS  Google Scholar 

  • Nieboer C, Boorsma DM, Woerdeman MJ, Kalsbeek GL (1980) Epidermolysis bullosa acquisita: immunofluorescence, electron microscopic and immunoelectron microscopic studies in four patients. Br J Dermatol 102:383–392

    Article  PubMed  CAS  Google Scholar 

  • Paller AS, Queen LL, Woodley DT, Lane AT, Gammon WR, Briggaman RA (1986) Organ specific, phylogenic and ontogenetic distribution of the epidermolysis bullosa acquisita antigen. J Invest Dermatol 86:376–379

    Article  PubMed  CAS  Google Scholar 

  • Park SB, Cho KH, Youn JL, Hwang DH, Kim SC, Chung JH (1997) Epidermolysis bullosa aquisita in childhood — a case mimicking chronic bullous dermatosis of childhood. Clin Exp Dermatol 22:220–222

    Article  PubMed  CAS  Google Scholar 

  • Ray TL, Levine JB, Weiss W, Ward PA (1982) Epidermolysis bullosa acquisita and inflammatory bowel disease. J Am Acad Dermatol 6:242–252

    Article  PubMed  CAS  Google Scholar 

  • Richter BJ, McNutt NS (1979) The spectrum of epidermolysis bullosa acquisita. Arch Dermatol 115:1325–1328

    Article  PubMed  CAS  Google Scholar 

  • Roenigk HH, Ryan JG, Bergfeld WF (1971) Epidermolysis bullosa acquisita: Report of three cases and review of all published cases. Arch Dermatol 103:1–10

    Article  PubMed  Google Scholar 

  • Rook AH, Jegasothy BV, Heald P, Nahass GT, Ditre C, Witmer WK, Lazarus GS, Edelson RL (1990) Extracoporeal photochemotherapy for drug-resistant pemphigus vulgaris. Ann Intern Med 112:303–305

    PubMed  CAS  Google Scholar 

  • Rusenko KW, Gammon WR, and Briggaman RA (1989) Type VII collagen is the antigen recognized by IgA anti-sub lamina densa autoantibodies. J Invest Dermatol 92:510 Abstract

    Article  Google Scholar 

  • Saha M, Cutler T, Bhogal B, Black MM, Groves RW (2009) Refractory epidermolysis bullosa acquisita: successful treatment with rituximab. Clin Exp Dermatol 34: e979–e980

    Article  PubMed  CAS  Google Scholar 

  • Sakai LY, Keene DR, Morris NP, Burgeson RE (1986) Type VII collagen is a major structural component of anchoring fibrils. J Cell Biol 103:1577–1586

    Article  PubMed  CAS  Google Scholar 

  • Schattenkirchner S, Eming S, Hunzelmann N, Krieg T, Smola H (1999) Treatment of epidermolysis bullosa acquisita with mycophenolate mofetil and autologous keratinocyte grafting. Br J Dermatol 141:932–933

    Article  PubMed  CAS  Google Scholar 

  • Schaumburg-Lever G, Rule A, Schmidt-Ullrich B, Lever WF (1975) Ultrastructural localization of in vivo bound immunoglobulins in bullous pemphigoid — A prelimnary report. J Invest Dermatol 64:47–49

    Article  PubMed  CAS  Google Scholar 

  • Schmidt E, Benoit S, Brocker EB, Zillikens D, Goebeler M. (2006) Successful adjuvant treatment of recalcitrant epidermolysis bullosa acquisita with anti-CD20 antibody rituximab. Arch Dermatol 142:147–150

    Article  PubMed  Google Scholar 

  • Sitaru C, Mihai S, Otto C, Chiriac MT, Hausser I, Dotterweich B, Saito H, Rose C, Ishiko A, Zillikens D (2005) Induction of dermal-epidermal separation in mice by passive transfer of antibodies specific to type VII collagen. J Clin Invest 2005, 115:870–878

    PubMed  CAS  Google Scholar 

  • Stewart MI, Woodley DT, Briggaman RA (1991) Acquired epidermolysis bullosa and associated symptomatic esophageal webs. Arch Dermatol 127:373–377

    Article  PubMed  CAS  Google Scholar 

  • Tanaka H, Ishida-Yamamoto A, Hashimoto T (1997) A novel variant of acquired epidermolysis bullosa with autoantibodies against the central triple-helical domain of type VII collagen. Lab Invest 77:623–632

    PubMed  CAS  Google Scholar 

  • Taniuchi K, Inaoki M, Nishimura Y, Mori T, Takehara K (1997) Nonscarring inflammatory epidermolysis bullosa acquisita with esophageal involvement and linear IgG deposits. J Am Acad Dermatol 36:320–322

    Article  PubMed  CAS  Google Scholar 

  • Tokuda Y, Amagai M, Yaoita H, Kawachi S, Ito T, Matsuyama I, Tsuchiya S, Saida T (1998) A case of inflammatory variant of epidermolysis bullosa acquisita: Chronic bullous dermatosis associated with non scarring mucosal blisters and circulating IgG anti-type-VII-collagen antibody. Dermatology 197:58–61

    Article  PubMed  CAS  Google Scholar 

  • Uitto J, Christiano AM (1994) Molecular basis for the dystrophic forms of epidermolysis bullosa: mutations in the type VII collagen gene. Arch Dermatol Res 287:16–22

    Article  PubMed  CAS  Google Scholar 

  • Vodegel RM, Kiss M, De Jong MCJM, Pas HH, Altmayer A, Molnar K, Husz S, Van Der Meer JB, Jonkman MF (1998) The use of skin substrate deficient in basement membrane molecules for the diagnosis of subepidermal autoimmune bullous disease. Eur J Dermatol 8:83–85

    PubMed  CAS  Google Scholar 

  • Wieme N, Lambert J, Moerman M, Geerts ML, Temmerman L, Naeyaert JM (1999) Epidermolysis bullosa acquisita with combined features of bullous pemphigoid and cicatricial pemphigoid. Dermatology 198:310–313

    Article  PubMed  CAS  Google Scholar 

  • Woodley DT, Sauder D, Talley MJ, Silver M, Grotendorst G, Qwarnstrom E (1983) Localization of basement membrane components after DEJ separation. J Invest Dermatol 81:149–153

    Article  PubMed  CAS  Google Scholar 

  • Woodley DT, Briggaman RA, O’Keefe EJ, Inman AO, Queen LL, Gammon WR (1984) Identification of the skin basement-membrane autoantigen in epidermolysis bullosa acquisita. N Engl J Med 310:1007–1015

    Article  PubMed  CAS  Google Scholar 

  • Woodley DT, O’Keefe EJ, Reese MJ, Mechanic GL, Briggaman RA, Gammon WR (1986) Epidermolysis bullosa acquisita antigen, a new major component of cutaneous basement membrane, is a glycoprotein with collagenous domains. J Invest Dermatol 86:668–672

    Article  PubMed  CAS  Google Scholar 

  • Woodley DT (1988) Epidermolysis bullosa acquisita. Prog Dermatol 22:1

    Google Scholar 

  • Woodley DT, Burgeson RE, Lunstrum G, Bruckner-Tuderman L, Reese MJ, Briggaman RA (1988) The epidermolysis bullosa acquisita antigen is the globular carboxyl terminus of type VII procollagen. J Clin Invest 81:683–687

    Article  PubMed  CAS  Google Scholar 

  • Woodley DT, Gammon WR, Briggaman RA (1998) Epidermolysis bullosa acquisita. In: Fitzpatrick TB, Eisen AZ, Wolff K, Freedberg IM, Austen KF, editors. Dermatology in general medicine. 4th ed. Vols. I–II. McGraw-Hill, New York, 702–709

    Google Scholar 

  • Woodley DT, Chang C, Saadat P, Ram R, Liu Z, Chen M (2005) 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 124:958–964

    Article  PubMed  CAS  Google Scholar 

  • Woodley DT, Ram R, Doostan A, Bandyopadhyay P, Huang Y, Remington J, Hou YP, Keene DR, Liu Z, Chen M (2006) Induction of epidermolysis bullosa acquisita in mice by passive transfer of autoantibodies from patients. J Invest Dermatol 126:1324–1330

    Article  Google Scholar 

  • Yancey KB. Cicatricial pemphigoid. In: Fitzpatrick TB, Eisen AZ, Wolff K, Freedberg IM, Austen KF (1998) Dermatology in general medicine. 4th ed. Vols. I–II. McGraw-Hill, New York, Inc., pp 674–679

    Google Scholar 

  • Yaoita H, Briggaman A, Lawley TJ, Provost TT, Katz SI (1981) Epidermolysis bullosa acquisita: Ultrastructural and immunological studies. J Invest Dermatol 76:288–292

    Article  PubMed  CAS  Google Scholar 

  • Zillikens D, Wever S, Roth A, Hashimoto T, Brocker EB (1995) Incidence of autoimmune subepidermal blistering dermatoses in a region of central Germany. Arch Dermatol 131:957–958

    Article  PubMed  CAS  Google Scholar 

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  1. Division of Dermatology LAC and USC Medical Center, 8th Floor General Hospital, 1200 North State Street, Los Angeles, California, 90033, USA

    David T. Woodley MD

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  1. Gene Kim
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Michael Hertl

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© 2011 Springer-Verlag/Wien

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Kim, G., Chen, M., Hallel-Halevy, D., Woodley, D.T. (2011). Epidermolysis Bullosa Acquisita. In: Hertl, M. (eds) Autoimmune Diseases of the Skin. Springer, Vienna. https://doi.org/10.1007/978-3-211-99225-8_6

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  • DOI: https://doi.org/10.1007/978-3-211-99225-8_6

  • Publisher Name: Springer, Vienna

  • Print ISBN: 978-3-211-99224-1

  • Online ISBN: 978-3-211-99225-8

  • eBook Packages: MedicineMedicine (R0)

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