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Vol. 220, No. 3, 2010
Issue release date: April 2010

Linear IgA Disease: Successful Application of Immunoadsorption and Review of the Literature

Kasperkiewicz M. · Meier M. · Zillikens D. · Schmidt E.
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Abstract

Linear IgA disease (LAD) is an autoimmune subepidermal blistering disorder characterized by IgA autoantibodies at the dermal-epidermal junction. Conventional first-line treatments include dapsone with or without oral glucocorticosteroids. Various other therapeutic approaches have been used in refractory patients. Immunoadsorption (IA) has been previously successfully applied in severe and/or otherwise treatment-resistant IgG-mediated immunobullous disorders. Here, we report a patient with a severe generalized LAD in whom adjuvant tryptophan IA was associated with rapid healing of skin lesions. Our observation suggests that IA may also be a helpful adjuvant treatment option for severe LAD.



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References

  1. Chorzelski TP, Jablonska S, Beutner EH: Linear IgA bullous dermatosis; in Beutner TP, Chorzelski SF, Bean S (eds): Immunopathology of the Skin. New York, Wiley & Sons, 1979, pp 315–323.
  2. Guide SV, Marinkovich MP: Linear IgA bullous dermatosis. Clin Dermatol 2001;19:719–727.
  3. Schmidt E, Klinker E, Opitz A, Herzog S, Sitaru C, Goebeler M, Mansouri-Taleghoni B, Bröcker EB, Zillikens D: Protein A immunoadsorption: a novel and effective adjuvant treatment of severe pemphigus. Br J Dermatol 2003;148:1222–1229.
  4. Eming R, Rech J, Barth S, Kalden JR, Schuler G, Harrer T, Hertl M: Prolonged clinical remission of patients with severe pemphigus upon rapid removal of desmoglein-reactive autoantibodies by immunoadsorption. Dermatology 2006;212:177–187.
  5. Zillikens D, Derfler K, Eming R, Fierlbeck G, Goebeler M, Hertl M, Hofmann SC, Karlhofer F, Kautz O, Nitschke M, Opitz A, Quist S, Rose C, Schanz S, Schmidt E, Shimanovich I, Michael M, Ziller F: Recommendations for the use of immunoapheresis in the treatment of autoimmune bullous diseases. J Dtsch Dermatol Ges 2007;5:881–887.
  6. Pfütze M, Eming R, Kneisel A, Kuhlmann U, Hoyer J, Hertl M: Clinical and immunological follow-up of pemphigus patients on adjuvant treatment with immunoadsorption or rituximab. Dermatology 2009;218:237–245.
  7. Schmidt E, Zillikens D: Immunoadsorption treatment for autoimmune bullous diseases; in Braun N (ed): Fundamentals and Applications of Immunoadsorption. Bremen, Uni-Med, 2009.
  8. Kromminga A, Scheckenbach C, Georgi M, Hagel C, Arndt R, Christophers E, Bröcker EB, Zillikens D: Patients with bullous pemphigoid and linear IgA disease show a dual IgA and IgG autoimmune response to BP180. J Autoimmun 2000;15:293–300.
  9. Zillikens D, Herzele K, Georgi M, Schmidt E, Chimanovitch I, Schumann H, Mascaro JM Jr, Diaz LA, Bruckner-Tuderman L, Bröcker EB, Giudice GJ: Autoantibodies in a subgroup of patients with linear IgA disease react with the NC16A domain of BP180. J Invest Dermatol 1999;113:947–953.
  10. Herrero-González JE, Sitaru C, Klinker E, Brocker EB, Zillikens D: Successful adjuvant treatment of severe bullous pemphigoid by tryptophan immunoadsorption. Clin Exp Dermatol 2005;30:519–522.
  11. Lüftl M, Stauber A, Mainka A, Klingel R, Schuler G, Hertl M: Successful removal of pathogenic autoantibodies in pemphigus by immunoadsorption with a tryptophan-linked polyvinylalcohol adsorber. Br J Dermatol 2003;149:598–605.
  12. Rao CR, Hall RP: Linear IgA dermatosis and chronic bullous disease of childhood; in Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ (eds): Fitzpatrick’s Dermatology in General Medicine, ed 7. New York, McGraw-Hill, 2008, pp 485–490.
  13. Wojnarowska F, Whitehead P, Leigh IM, Bhogal BS, Black MM: Identification of the target antigen in chronic bullous disease of childhood and linear IgA disease of adults. Br J Dermatol 1991;124:157–162.
  14. Marinkovich MP, Taylor TB, Keene DR, Burgeson RE, Zone JJ: LAD-1, the linear IgA bullous dermatosis autoantigen, is a novel 120-kDa anchoring filament protein synthesized by epidermal cells. J Invest Dermatol 1996;106:734–738.
  15. Zone JJ, Taylor TB, Meyer LJ, Petersen MJ: The 97 kDa linear IgA bullous disease antigen is identical to a portion of the extracellular domain of the 180 kDa bullous pemphigoid antigen, BPAg2. J Invest Dermatol 1998;110:207–210.
  16. Zone JJ, Taylor TB, Kadunce DP, Meyer LJ: Identification of the cutaneous basement membrane zone antigen and isolation of antibody in linear immunoglobulin A bullous dermatosis. J Clin Invest 1990;85:812–820.
  17. Pas HH, Kloosterhuis GJ, Heeres K, van der Meer JB, Jonkman MF: Bullous pemphigoid and linear IgA dermatosis sera recognize a similar 120-kDa keratinocyte collagenous glycoprotein with antigenic cross-reactivity to BP180. J Invest Dermatol 1997;108:423–429.
  18. Hirako Y, Usukura J, Uematsu J, Hashimoto T, Kitajima Y, Owaribe K: Cleavage of BP180, a 180-kDa bullous pemphigoid antigen, yields a 120-kDa collagenous extracellular polypeptide. J Biol Chem 1998;273:9711–9717.
  19. Schäcke H, Schumann H, Hammami- Hauasli N, Raghunath M, Bruckner-Tuderman L: Two forms of collagen XVII in keratinocytes: a full-length transmembrane protein and a soluble ectodomain. J Biol Chem 1998;273:25937–25943.
  20. Hirako Y, Nishizawa Y, Sitaru C, Opitz A, Marcus K, Meyer HE, Butt E, Owaribe K, Zillikens D: The 97-kDa (LABD97) and 120-kDa (LAD-1) fragments of bullous pemphigoid antigen 180/type XVII collagen have different N-termini. J Invest Dermatol 2003;121:1554–1556.
  21. Franzke CW, Bruckner-Tuderman L, Blobel CP: Shedding of collagen XVII/BP180 in skin depends on both ADAM10 and ADAM9. J Biol Chem 2009;284:23386–23396.
  22. Zambruno G, Manca V, Kanitakis J, Cozzani E, Nicolas JF, Giannetti A: Linear IgA bullous dermatosis with autoantibodies to a 290 kD antigen of anchoring fibrils. J Am Acad Dermatol 1994;31:884–888.
  23. Ghohestani RF, Nicolas JF, Kanitakis J, Claudy A: Linear IgA bullous dermatosis with IgA antibodies exclusively directed against the 180- or 230-kDa epidermal antigens. J Invest Dermatol 1997;108:854–858.
  24. Natsuga K, Nishie W, Shinkuma S, Moriuchi R, Shibata M, Nishimura M, Hashimoto T, Shimizu H: Circulating IgA and IgE autoantibodies in antilaminin-332 mucous membrane pemphigoid. Br J Dermatol DOI: 10.1111/j.1365–2133.2009.09508.x.

    External Resources

  25. Zillikens D, Herzele K, Georgi M, Schmidt E, Chimanovitch I, Schumann H, Mascaro JM Jr, Diaz LA, Bruckner-Tuderman L, Bröcker EB, Giudice GJ: Autoantibodies in a subgroup of patients with linear IgA disease react with the NC16A domain of BP180. J Invest Dermatol 1999;113:947–953.
  26. Schmidt E, Herzele K, Schumann H, Wesselmann U, Chimanovitch I, Bruckner-Tuderman L, Bröcker EB, Giudice GJ, Zillikens D: Linear IgA disease with circulating IgA antibodies against the NC16A domain of BP180. Br J Dermatol 1999;140:964–966.
  27. Gläser R, Sticherlin M: Successful treatment of linear IgA bullous dermatosis with mycophenolate mofetil. Acta Derm Venereol 2002;82:308–309.
  28. Farley-Li J, Mancini AJ: Treatment of linear IgA bullous dermatosis of childhood with mycophenolate mofetil. Arch Dermatol 2003;139:1121–1124.
  29. Talhari C, Mahnke N, Ruzicka T, Megahed M: Successful treatment of linear IgA disease with mycophenolate mofetil as a corticosteroid sparing agent. Clin Exp Dermatol 2005;30:297–298.
  30. Lewis MA, Yaqoob NA, Emanuel C, Potts AJ: Successful treatment of oral linear IgA disease using mycophenolate. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;103:483–486.
  31. Marzano AV, Ramoni S, Spinelli D, Alessi E, Berti E: Refractory linear IgA bullous dermatosis successfully treated with mycophenolate sodium. J Dermatol Treat 2008;19:364–367.
  32. Aram H: Linear IgA bullous dermatosis: successful treatment with colchicine. Arch Dermatol 1984;120:960–961.
  33. Zeharia A, Hodak E, Mukamel M, Danziger Y, Mimouni M: Successful treatment of chronic bullous dermatosis of childhood with colchicine. J Am Acad Dermatol 1994;30:660–661.
  34. Banodkar DD, al-Suwaid AR: Colchicine as a novel therapeutic agent in chronic bullous dermatosis of childhood. Int J Dermatol 1997;36:213–216.
  35. Ang P, Tay YK: Treatment of linear IgA bullous dermatosis of childhood with colchicine. Pediatr Dermatol 1999;16:50–52.
  36. Benbenisty KM, Bowman PH, Davis LS: Localized linear IgA disease responding to colchicine. Int J Dermatol 2002;41:56–58.
  37. Hernández-Machín B, Peñate Y, Báez B, Borrego L: Linear IgA bullous dermatosis of adults treated with colchicine. Actas Dermosifiliogr 2006;97:549–550.
  38. Burrows NP, Jones RR: Methotrexate and cyclosporin are of value in the treatment of adult linear IgA disease. J Dermatol Treat 1992;3:31–33.

    External Resources

  39. Young HS, Coulson IH: Linear IgA disease: successful treatment with cyclosporine. Br J Dermatol 2000;143:204–205.
  40. Khan IU, Bhol KC, Ahmed AR: Linear IgA bullous dermatosis in a patient with chronic renal failure: response to intravenous immunoglobulin therapy. J Am Acad Dermatol 1999;40:485–488.
  41. Letko E, Bhol K, Foster CS, Ahmed AR: Linear IgA bullous disease limited to the eye: a diagnostic dilemma: response to intravenous immunoglobulin therapy. Ophthalmology 2000;107:1524–1528.
  42. Kroiss MM, Vogt T, Landthaler M, Stolz W: High-dose intravenous immune globulin is also effective in linear IgA disease. Br J Dermatol 2000;142:582.
  43. Goebeler M, Seitz C, Rose C, Sitaru C, Jeschke R, Marx A, Bröcker EB, Zillikens D: Successful treatment of linear IgA disease with salazosulphapyridine and intravenous immunoglobulins. Br J Dermatol 2003;149:912–914.
  44. Cauza K, Hinterhuber G, Sterniczky B, Brugger K, Pieczkowski F, Karlhofer F, Wolff K, Foedinger D: Unusual clinical manifestation of linear IgA dermatosis: a report of two cases. J Am Acad Dermatol 2004;51(suppl):S112–S117.

    External Resources

  45. Gluth MB, Witman PM, Thompson DM: Upper aerodigestive tract complications in a neonate with linear IgA bullous dermatosis. Int J Pediatr Otorhinolaryngol 2004;68:965–970.
  46. Segura S, Iranzo P, Martínez-de Pablo I, Mascaró JM Jr, Alsina M, Herrero J, Herrero C: High-dose intravenous immunoglobulins for the treatment of autoimmune mucocutaneous blistering diseases: evaluation of its use in 19 cases. J Am Acad Dermatol 2007;56:960–967.
  47. Piamphongsant T, Sirimachan S, Himmunknan P: Juvenile blistering diseases: the problems of diagnosis and treatment. Asian Pac J Allergy Immunol 1986;4:133–137.
  48. Pulimood S, Ajithkumar K, Jacob M, George S, Chandi SM: Linear IgA bullous dermatosis of childhood: treatment with dapsone and co-trimoxazole. Clin Exp Dermatol 1997;22:90–91.
  49. Peterson JD, Chan LS: Linear IgA bullous dermatosis responsive to trimethoprim-sulfamethoxazole. Clin Exp Dermatol 2007;32:756–758.
  50. Cooper SM, Powell J, Wojnarowska F: Linear IgA disease: successful treatment with erythromycin. Clin Exp Dermatol 2002;27:677–679.
  51. Farrant P, Darley C, Carmichael A: Is erythromycin an effective treatment for chronic bullous disease of childhood? A national survey of members of the British Society for Paediatric Dermatology. Pediatr Dermatol 2008;25:479–482.
  52. Denguezli M, Ben Nejma B, Nouira R, Korbi S, Bardi R, Ayed K, Essoussi AS, Jomaa B: IgA linear bullous dermatosis in children: a series of 12 Tunisian patients. Ann Dermatol Venereol 1994;121:888–892.
  53. Alajlan A, Al-Khawajah M, Al-Sheikh O, Al-Saif F, Al-Rasheed S, Al-Hoqail I, Hamadah IR: Treatment of linear IgA bullous dermatosis of childhood with flucloxacillin. J Am Acad Dermatol 2006;54:652–656.
  54. Skinner RB Jr, Rotondo CK, Schneider MA, Raby L, Rosenberg EW: Treatment of chronic bullous dermatosis of childhood with oral dicloxacillin. Pediatr Dermatol 1995;12:65–66.
  55. Siegfried EC, Sirawan S: Chronic bullous disease of childhood: successful treatment with dicloxacillin. J Am Acad Dermatol 1998;39:797–800.
  56. Peoples D, Fivenson DP: Linear IgA bullous dermatosis: successful treatment with tetracycline and nicotinamide. J Am Acad Dermatol 1992;26:498–499.
  57. Chaffins ML, Collison D, Fivenson DP: Treatment of pemphigus and linear IgA dermatosis with nicotinamide and tetracycline: a review of 13 cases. J Am Acad Dermatol 1993;28:998–1000.
  58. Yomada M, Komai A, Hashimato T: Sublamina densa-type linear IgA bullous dermatosis successfully treated with oral tetracycline and niacinamide. Br J Dermatol 1999;141:608–609.
  59. Khanna N, Pandhi RK, Gupta S, Singh MK: Response of chronic bullous dermatosis of childhood to a combination of dapsone and nicotinamide. J Eur Acad Dermatol Venereol 2001;15:368.
  60. Zone JJ, Egan CA, Taylor TB, Meyer LJ: IgA autoimmune disorders: development of a passive transfer mouse model. J Invest Dermatol Symp Proc 2004;9:47–51.
  61. Matic G, Hofmann D, Winkler R, Tiess M, Michelsen A, Schneidewind JM, Hebestreit G, Keysser M, Müller W, Kinze EM, Ramlow W: Removal of immunoglobulins by protein A versus an antihuman immunoglobulin G-based system: evaluation of 602 sessions of extracorporeal immunoadsorption. Artif Organs 2000;24:103–107.
  62. Hirata N, Kuriyama T, Yamawaki N: Immusorba TR and PH. Ther Apher Dial 2003;7:85–90.
  63. Tsuboi Y, Takahashi M, Ishikawa Y, Okada H, Yamada T: Elevated bradykinin and decreased carboxypeptidase R as a cause of hypotension during tryptophan column immunoabsorption therapy. Ther Apher 1998;2:297–299.


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