Skip to main content
SpringerLink
Log in

Autoimmune Bullous Dermatoses in the Elderly

An Update on Pathophysiology, Diagnosis and Management

  • Review Article
  • Published:
Drugs & Aging Aims and scope Submit manuscript

Abstract

Elderly individuals are susceptible to autoimmune bullous dermatoses (ABDs), which may be associated with high morbidity and mortality. ABDs result from an autoimmune response to components of the basement membrane zone at the dermal-epidermal junction or desmosomes.

Bullous pemphigoid results from autoimmunity to hemidesmosomal proteins present in the basement membrane of stratified squamous epithelia. Patients present with tense blisters in flexural areas of the skin. Mild disease may be treated with potent topical corticosteroids, while extensive disease usually requires systemic corticosteroids or systemic immunosuppressive agents such as azathioprine. Mucosal pemphigoid affects one or more mucous membranes that are lined by stratified squamous epithelia. The two most commonly involved sites are the eye and the oral cavity. Lesions frequently result in scar formation that may cause blindness. Patients with severe disease or ocular involvement require aggressive therapy with corticosteroids and cyclophosphamide.

Epidermolysis bullosa acquisita results from autoimmunity to type VII collagen in the anchoring fibrils of the basement membrane. Lesions may either arise on an inflammatory base or be non-inflammatory and result primarily from trauma. Treatment options include corticosteroids, dapsone, ciclosporin, methotrexate and plasmapheresis/immunoapheresis.

Paraneoplastic pemphigus results from autoimmunity to multiple desmosomal antigens. The disorder is associated with neoplasms, especially leukaemia, lymphoma and thymoma. Patients present with stomatitis and polymorphous skin eruption. The disease may respond to successful treatment of the underlying neoplasm or may require immunosuppressive therapy.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Table I
Table II
Table III
Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9

Similar content being viewed by others

References

  1. Mutasim DF. Autoimmune bullous dermatoses in the elderly: diagnosis and management. Drugs Aging 2003; 20(9): 663–81

    Article  PubMed  Google Scholar 

  2. Grove GL, Duncan S, Kligman AM. Effect of aging on the blistering of human skin with ammonium hydroxide. Br J Dermatol 1982; 107: 383–400

    Article  Google Scholar 

  3. Montagna W, Carlisle K. Structural changes in aging human skin. J Invest Dermatol 1979; 73: 47–53

    Article  PubMed  CAS  Google Scholar 

  4. Dubey DP, Yunis EJ. Aging and nutritional effects on immune functions in humans. In: Stites DP, Terr AI, editors. Basic and clinical immunology. 7th ed. Norwalk (CT): Appleton and Lange, 1991: 190–3

    Google Scholar 

  5. Burgeson RE, Christiano AM. The dermal-epidermal junction. Curr Opin Cell Biol 1998; 9: 651–8

    Article  Google Scholar 

  6. Roscoe JT, Diaz L, Sampaio SA, et al. Brazilian pemphigus foliaceus autoantibodies are pathogenic to BALB/c mice by passive transfer. J Invest Dermatol 1985; 85: 538–41

    Article  PubMed  CAS  Google Scholar 

  7. Anhalt GJ, Labib RS, Voorhees JJ, et al. Induction of pemphigus in neonatal mice by passive transfer of IgG from patients with the disease. N Engl J Med 1982; 306: 1189–96

    Article  PubMed  CAS  Google Scholar 

  8. Amagai M, Klaus-Kovtun V, Stanley JR. Autoantibodies against a novel epithelial cadherin in pemphigus vulgaris, a disease of cell adhesion. Cell 1991; 67: 869–77

    Article  PubMed  CAS  Google Scholar 

  9. Beutner EH, Jordon RE. Demonstration of skin antibodies in sera of pemphigus vulgaris patients by indirect immunofluorescent staining. Proc Soc Exp Biol Med 1964; 117: 505–10

    PubMed  CAS  Google Scholar 

  10. Rappersberger K, Roos N, Stanley JR. Immunomorphological and biochemical identification of the pemphigus foliaceus autoantigen within desmosomes. J Invest Dermatol 1992; 99: 323–30

    Article  PubMed  CAS  Google Scholar 

  11. Karpati S, Amagai M, Prussick R, et al. Pemphigus vulgaris antigen, a desmoglein type of cadherin, is localized within keratinocyte desmosomes. J Cell Biol 1993; 122: 409–15

    Article  PubMed  CAS  Google Scholar 

  12. Stanley JR. Cell adhesion molecules as targets of autoantibodies in pemphigus and pemphigoid, bullous diseases due to defective epidermal cell adhesion. Adv Immunol 1993; 53: 291–325

    Article  PubMed  CAS  Google Scholar 

  13. Eyre RW, Stanley JR. Identification of pemphigus vulgaris antigen extracted from normal human epidermis and comparison with pemphigus foliaceus antigen. J Clin Invest 1988; 81: 807–12

    Article  PubMed  CAS  Google Scholar 

  14. Hu CH, Michel B, Schlitz JR. Epidermal acantholysis induced in vitro by pemphigus autoantibody. Am J Pathol 1978; 90: 345–51

    PubMed  CAS  Google Scholar 

  15. Diaz LA, Giudice GJ. End of the century overview of skin blisters. Arch Dermatol 2000; 136: 106–12

    Article  PubMed  CAS  Google Scholar 

  16. Stanley JR. Pemphigus and pemphigoid as paradigms of organ-specific, autoantibody-mediated diseases. J Clin Invest 1989; 83: 1443–8

    Article  PubMed  CAS  Google Scholar 

  17. Liu Z, Giudice GJ, Zhou X, et al. A major role for neutrophils in experimental bullous pemphigoid. J Clin Invest 1997; 100: 1256–63

    Article  PubMed  CAS  Google Scholar 

  18. Liu Z, Diaz LA, Troy JL, et al. A passive transfer model of the organ-specific autoimmune disease, bullous pemphigoid, using antibodies generated against the hemidesmosomal antigen, BP 180. J Clin Invest 1993; 92: 2480–8

    Article  PubMed  CAS  Google Scholar 

  19. Sitaru C, Mihai S, Otto C, et al. Induction of dermal-epidermal separation in mice by passive transfer of antibodies specific to type VII collagen. J Clin Invest 2005 Apr; 115(4): 870–8

    PubMed  CAS  Google Scholar 

  20. Sitaru C, Zillikens D. Mechanisms of blister induction by autoantibodies. Exp Dermatol 2005 Dec; 14(12): 861–75

    Article  PubMed  CAS  Google Scholar 

  21. Lazarova Z, Yee C, Darling T, et al. Passive transfer of anti-laminin 5 antibodies induces subepidermal blisters in neonatal mice. J Clin Invest 1996 Oct 1; 98(7): 1509–18

    Article  PubMed  CAS  Google Scholar 

  22. Wolverton SE. Comprehensive dermatologic drug therapy. Philadelphia (PA): WB Saunders Company, 2001

    Google Scholar 

  23. Hardman JG, Limbird LE, Gilman AG. Goodman & Gilman’s the pharmacological basis of therapeutics. 10th ed. New York: McGraw-Hill, 2001

    Google Scholar 

  24. Berk MA, Lorincz AL. The treatment of bullous pemphigoid with tetracycline and niacinamide. Arch Dermatol 1986; 122: 670–4

    Article  PubMed  CAS  Google Scholar 

  25. Esterly NB, Furey NL, Flanagan LE. The effect of antimicrobial agents on leukocyte chemotaxis. J Invest Dermatol 1978; 70: 51–5

    Article  PubMed  CAS  Google Scholar 

  26. Humbert P, Treffel P, Chapuis JF, et al. The tetracyclines in dermatology. J Am Acad Dermatol 1991; 25: 691–7

    Article  PubMed  CAS  Google Scholar 

  27. Kazatchkine MD, Kaveri SV. Immunomodulation of autoimmune and inflammatory diseases with intravenous immune globulin. N Engl J Med 2001 Sep 6; 345(10): 747–55

    Article  PubMed  CAS  Google Scholar 

  28. Li N, Zhao M, Hilario-Vargas J, et al. Complete FcRn dependence for intravenous Ig therapy in autoimmune skin blistering diseases. J Clin Invest 2005 Dec; 115(12): 3440–50

    Article  PubMed  CAS  Google Scholar 

  29. Sesarman A, Sitaru AG, Olaru F, et al. Neonatal Fc receptor deficiency protects from tissue injury in experimental epidermolysis bullosa acquisita. J Mol Med 2008 Aug;86(8):951–9

    Article  PubMed  CAS  Google Scholar 

  30. Eisenberg R, Looney RJ. The therapeutic potential of anti-CD20 “what do B-cells do?” Clin Immunol 2005 Dec; 117(3): 207–13

    Article  PubMed  CAS  Google Scholar 

  31. Schmidt E, Hunzelmann N, Zillikens D, et al. Rituximab in refractory autoimmune bullous diseases. Clin Exp Dermatol 2006 Jul; 31(4): 503–8

    Article  PubMed  CAS  Google Scholar 

  32. Martin F, Chan AC. B cell immunobiology in disease: evolving concepts from the clinic. Annu Rev Immunol 2006; 24: 467–96

    Article  PubMed  CAS  Google Scholar 

  33. Kazkaz H, Isenberg D. Anti B cell therapy (rituximab) in the treatment of autoimmune diseases. Curr Opin Pharmacol 2004 Aug; 4(4): 398–402

    Article  PubMed  CAS  Google Scholar 

  34. Zillikens D, Derfler K, Eming R, et al. Recommendations for the use of immunoapheresis in the treatment of autoimmune bullous diseases. J Dtsch Dermatol Ges 2007; 5: 881–7

    Article  PubMed  Google Scholar 

  35. Rook A, Wilkinson DS, Ebling FJG. Textbook of dermatology. Oxford: Blackwell Scientific Publications, 1979

    Google Scholar 

  36. Bean SF. Bullous pemphigoid. In: Ahmed AR, editor. Clinical dermatology. Philadelphia (PA): JB Lippincott, 1987: 13–7

    Google Scholar 

  37. Marsden RA, McKee PH, Bhogal B, et al. A study of benign chronic bullous dermatosis of childhood and comparison with dermatitis herpetiformis and bullous pemphigoid occurring in childhood. Clin Exp Dermatol 1980; 5: 159–72

    Article  PubMed  CAS  Google Scholar 

  38. Jordon RE. Bullous pemphigoid, cicatricial pemphigoid, and chronic bullous dermatosis of childhood. In: Fitzpatrick TB, Eisen AZ, Wolff K, et al., editors. Dermatology in general medicine. 3rd ed. New York: McGraw-Hill, Inc., 1987: 580–6

    Google Scholar 

  39. Sison-Fonacier L, Bystryn JC. Regional variations in antigenic properties of skin: a possible cause for disease-specific distribution of skin lesions. J Exp Med 1986; 164: 2125–30

    Article  PubMed  CAS  Google Scholar 

  40. Downham TF, Chapel TA. Bullous pemphigoid: therapy in patients without diabetes mellitus. Arch Dermatol 1978; 114: 1639–42

    Article  PubMed  Google Scholar 

  41. Provost TT, Maize JC, Ahmed AR, et al. Unusual sub-epidermal bullous diseases with immunologic features of bullous pemphigoid. Arch Dermatol 1979; 115: 156–60

    Article  PubMed  CAS  Google Scholar 

  42. Ahmed AR, Newcomer VD. Bullous pemphigoid. In: Ahmed AR, editor. Clinical dermatology. Philadelphia (PA): JB Lippincott, 1987: 6–12

    Google Scholar 

  43. Ahmed AR, Maize JC, Provost TT. Bullous pemphigoid: clinical and immunologic follow-up after successful therapy. Arch Dermatol 1977; 113: 1043–6

    Article  PubMed  CAS  Google Scholar 

  44. Bingham EA, Burrow D, Sanford JC. Prolonged pruritus and bullous pemphigoid. Clin Exp Dermatol 1984; 9: 564–70

    Article  PubMed  CAS  Google Scholar 

  45. Lever WF. Pemphigus and pemphigoid. Springfield (IL): Charles C. Thomas, 1965

    Google Scholar 

  46. Korman N. Bullous pemphigoid. J Am Acad Dermatol 1987; 16: 907–24

    Article  PubMed  CAS  Google Scholar 

  47. Person JR, Rogers III RS. Bullous and cicatricial pemphigoid: clinical, histopathologic and immunopathologic correlations. Mayo Clin Proc 1977; 52: 54–66

    PubMed  CAS  Google Scholar 

  48. Parker SR, Dyson S, Brisman S, et al. Mortality of bullous pemphigoid: an evaluation of 223 patients and comparison with the mortality in the general population in the United States. J Am Acad Dermatol 2008 Oct; 59(4): 582–8

    Article  PubMed  Google Scholar 

  49. Langan SM, Smeeth L, Hubbard R, et al. Bullous pemphigoid and pemphigus vulgaris — incidence and mortality in the UK: population based cohort study [abstract]. BMJ 2008; 337: a180

    Article  PubMed  CAS  Google Scholar 

  50. Savin JA. Bullous pemphigoid. In: Ahmed AR, editor. Clinical dermatology. Philadelphia (PA): JB Lippincott, 1987: 52–9

    Google Scholar 

  51. Rzany B, Partscht K, Jung M, et al. Risk factors for lethal outcome in patients with bullous pemphigoid. Arch Dermatol 2002; 138: 903–8

    Article  PubMed  Google Scholar 

  52. Joly P, Roujeau J-C, Benichou J, et al. A comparison of oral and topical corticosteroids in patients with bullous pemphigoid. N Engl J Med 2002; 346: 321–7

    Article  PubMed  CAS  Google Scholar 

  53. Flotte TJ. Pathology of pemphigoid. Clin Dermatol 1987; 5: 71–80

    Article  PubMed  CAS  Google Scholar 

  54. Lever WF, Schaumburg-Lever G. Histopathology of the skin. 6th ed. Philadelphia (PA): JB Lippincott Co., 1983

    Google Scholar 

  55. Mutasim DF, Diaz LA. The relevance of immunohistochemical techniques in the differentiation of subepidermal bullous diseases. Am J Dermatopathol 1991; 13: 77–83

    Article  PubMed  CAS  Google Scholar 

  56. Church R. Pemphigoid treated with corticosteroids. Br J Dermatol 1960; 72: 431–41

    Google Scholar 

  57. Siegel J, Eaglstein WH. High-dose methylprednisolone in the treatment of bullous pemphigoid. Arch Dermatol 1984; 120: 1157–65

    Article  PubMed  CAS  Google Scholar 

  58. Greaves MW, Burton JL, Marks J, et al. Azathioprine in the treatment of bullous pemphigoid. BMJ 1971; 1: 144–5

    Article  PubMed  CAS  Google Scholar 

  59. Burton JL, Greaves MW. Azathioprine for pemphigus and pemphigoid: a 4 year follow-up. Br J Dermatol 1974; 91: 103–9

    Article  PubMed  CAS  Google Scholar 

  60. Ahmed AR, Moy R. Azathioprine. Int J Dermatol 1981; 20: 461–9

    Article  PubMed  CAS  Google Scholar 

  61. Krain LS, Landau JW, Newcomer VD. Cyclophosphamide in the treatment of pemphigus vulgaris and bullous pemphigoid. Arch Dermatol 1972; 106: 657–61

    Article  PubMed  CAS  Google Scholar 

  62. Ahmed AR, Hombal SM. Cyclophosphamide (cytoxan): a review of relevant pharmacology and clinical use. J Am Acad Dermatol 1984; 6: 1115–26

    Article  Google Scholar 

  63. Thivolet J, Barthelemy H, Rigot-Muller G, et al. Effects of cyclosporin on bullous pemphigoid and pemphigus. Lancet 1985; 1: 334–5

    Article  PubMed  CAS  Google Scholar 

  64. Person JR, Rogers III RS. Bullous pemphigoid responding to sulfapyridine and the sulfones. Arch Dermatol 1977; 113:610–5

    Article  PubMed  CAS  Google Scholar 

  65. Goldberg NS, Robinson JK, Roenigk Jr HH, et al. Plasmapheresis therapy for bullous pemphigoid. Arch Dermatol 1985; 121: 1484–5

    Article  PubMed  CAS  Google Scholar 

  66. Roujeau JC, Guillaume JC, Morel P, et al. Plasma exchange in bullous pemphigoid. Lancet 1984; 2: 486–9

    Article  PubMed  CAS  Google Scholar 

  67. Guillaume JC, Vaillant L, Bernard P, et al. Controlled trial of azathioprine and plasma exchange in addition to prednisolone in the treatment of bullous pemphigoid. Arch Dermatol 1993; 129: 49–53

    Article  PubMed  CAS  Google Scholar 

  68. Engineer L, Ahmed AR. Role of intravenous immunoglobulin in the treatment of bullous pemphigoid: analysis of current data. J Am Acad Dermatol 2001; 44: 83–8

    Article  PubMed  CAS  Google Scholar 

  69. Ahmed AR. Intravenous immunoglobulin therapy in the treatment of patients with pemphigus vulgaris unresponsive to conventional treatment. J Am Acad Dermatol 2001; 45: 679–90

    Article  PubMed  CAS  Google Scholar 

  70. Thomas I, Khorenian S, Arbesteld DM. Treatment of generalized bullous pemphigoid with oral tetracycline. J Am Acad Dermatol 1993; 28: 74–7

    Article  PubMed  CAS  Google Scholar 

  71. Fivenson DP, Breneman DL, Rosen GB, et al. Nicotinamide and tetracycline therapy of bullous pemphigoid. Arch Dermatol 1994; 130: 753–8

    Article  PubMed  CAS  Google Scholar 

  72. Khumalo N, Kirtschig G, Middleton P, et al. Interventions for bullous pemphigoid. Cochrane Database Syst Rev 2005 Jul 20; (3): CD002292

    Google Scholar 

  73. Wojnarowska F, Kirtschig G, Highet AS, et al. Guidelines for the management of bullous pemphigoid. Br J Dermatol 2002; 147: 214–21

    Article  PubMed  CAS  Google Scholar 

  74. Schmidt E, Seitz CS, Benoit S, et al. Rituximab in autoimmune bullous diseases: mixed responses and adverse effects. Br J Dermatol 2007 Feb; 156(2): 352–6

    Article  PubMed  CAS  Google Scholar 

  75. Schmidt E, Brocker EB, Goebeler M. Rituximab in treatment-resistant autoimmune blistering skin disorders. Clin Rev Allergy Immunol 2008 Feb; 34(1): 56–64

    Article  PubMed  CAS  Google Scholar 

  76. Jacoby Jr WD, Bartholome CW, Ramchand SC, et al. Ci-catricial pemphigoid (Brunsting-Perry type) case report and immunofluorescence findings. Arch Dermatol 1978; 114: 779–81

    Article  PubMed  Google Scholar 

  77. Michel B, Bean SF, Chorzelski T, et al. Cicatricial pemphigoid of Brunsting-Perry: immunofluorescent studies. Arch Dermatol 1977; 113: 1403–5

    Article  PubMed  CAS  Google Scholar 

  78. Brunsting LA, Perry HO. Benign pemphigoid: a report of seven cases with chronic, scarring, herpetiform plaques about the head and neck. Arch Dermatol 1957; 75: 489–501

    Article  CAS  Google Scholar 

  79. Fine J-D. Cicatricial pemphigoid. In: Wojnarowska F, Briggaman RA, editors. Management of blistering diseases. New York: Raven Press, 1990: 83–92

    Google Scholar 

  80. Fine J-D. Cicatricial and localized pemphigoid. In: Jordon RE, editor. Immunologic diseases of the skin. Norwalk (CT): Appleton and Lange, 1991: 303–13

    Google Scholar 

  81. Laskaris G, Sklavounou A, Stratigos J. Bullous pemphigoid, cicatricial pemphigoid and pemphigus vulgaris: a comparative clinical survey of 278 cases. Oral Surg Oral Med Oral Pathol 1982; 54: 656–62

    Article  PubMed  CAS  Google Scholar 

  82. Foster ME, Nally FF. Benign mucous membrane pemphigoid (cicatricial mucosal pemphigoid): a reconsideration. Oral Surg Oral Med Oral Pathol 1977; 44: 697–705

    Article  PubMed  CAS  Google Scholar 

  83. Leonard JN, Wright P, Williams DM, et al. The relationship between linear IgA disease and benign mucous membrane pemphigoid. Br J Dermatol 1984; 110: 307–14

    Article  PubMed  CAS  Google Scholar 

  84. Frith PA, Venning VA, Wojnarowska F, et al. Conjunctival involvement in cicatricial and bullous pemphigoid: a clinical and immunopathological study. Br J Ophthalmol 1989; 73: 52–6

    Article  PubMed  CAS  Google Scholar 

  85. Venning VA, Frith PA, Bron AJ, et al. Mucosal involvement in bullous and cicatricial pemphigoid: a clinical and immunopathological study. Br J Dermatol 1988; 118: 7–15

    Article  PubMed  CAS  Google Scholar 

  86. Behlen CH, Mackey DM. Benign mucous membrane pemphigus with a generalized eruption. Arch Dermatol 1965; 92: 566–7

    Article  PubMed  CAS  Google Scholar 

  87. Ackerman AB. Histologic diagnosis of inflammatory skin diseases. Philadelphia (PA): Lea and Febiger, 1978

    Google Scholar 

  88. Lever WF, Schaumburg-Lever G. Non-infectious vesicular and bullous diseases. In: Lever WF, Schaumburg-Lever G, editors. Histopathology of the skin. 7th ed. Philadelphia (PA): JB Lippincott Co., 1990: 103–51

    Google Scholar 

  89. Brauner GJ, Jimbow K. Benign mucous membrane pemphigoid: an unusual case with electron microscopic findings. Arch Dermatol 1972; 106: 535–40

    Article  PubMed  CAS  Google Scholar 

  90. Fine J-D, Neises GR, Katz SI. Immunofluorescence and immunoelectron microscopic studies in cicatricial pemphigoid. J Invest Dermatol 1984; 82: 39–43

    Article  PubMed  CAS  Google Scholar 

  91. Bean SF, Waisman M, Michel B, et al. Cicatricial pemphigoid: immunofluorescent studies. Arch Dermatol 1972; 106: 195–9

    Article  PubMed  CAS  Google Scholar 

  92. Fleming MG, Valenzuela R, Bergfeld WF, et al. Mucous gland basement membrane immunofluorescence in cicatricial pemphigoid. Arch Dermatol 1988; 124: 1407–10

    Article  PubMed  CAS  Google Scholar 

  93. Anhalt GJ, Morrison LH. Bullous and cicatricial pemphigoid. Autoimmunity 1991; 4: 17–35

    Article  CAS  Google Scholar 

  94. Thorne JE, Woreta FA, Jabs DA, et al. Treatment of ocular mucous membrane pemphigoid with immunosuppressive drug therapy. Ophthalmology 2008; 115: 2146–52

    Article  PubMed  Google Scholar 

  95. Rogers III RS, Seehafer JR, Perry HO. Treatment of cicatricial (benign mucous membrane) pemphigoid with dapsone. J Am Acad Dermatol 1982; 6: 215–23

    Article  PubMed  Google Scholar 

  96. Rogers III RS, Mehregan DA. Dapsone therapy of cicatricial pemphigoid. Semin Dermatol 1988; 7: 201–5

    PubMed  Google Scholar 

  97. Fern AI, Jay JL, Young H, et al. Dapsone therapy for the acute inflammatory phase of ocular pemphigoid. Br J Ophthalmol 1992; 76: 332–5

    Article  PubMed  CAS  Google Scholar 

  98. Elder MJ, Leonard J, Dart JK. Sulphapyridine: a new agent for the treatment of ocular cicatricial pemphigoid. Br J Ophthalmol 1996; 80: 549–52

    Article  PubMed  CAS  Google Scholar 

  99. Reiche L, Wojnarowska F, Mallon E. Combination therapy with nicotinamide and tetracyclines for cicatricial pemphigoid: further support for its efficacy. Clin Exp Dermatol 1998; 23: 254–7

    Article  PubMed  CAS  Google Scholar 

  100. Dragan L, Eng AM, Lam S, et al. Tetracycline and niacinamide: treatment alternatives in ocular cicatricial pemphigoid. Cutis 1999; 63: 181–3

    PubMed  CAS  Google Scholar 

  101. Poskitt L, Wojnarowska F. Minimizing cicatricial pemphigoid orodynia with minocycline. Br J Dermatol 1995; 132: 784–9

    Article  PubMed  CAS  Google Scholar 

  102. Brody HJ, Pirozzi DJ. Benign mucous membrane pemphigoid: response to therapy with cyclophosphamide. Arch Dermatol 1977; 113: 1598–9

    Article  PubMed  CAS  Google Scholar 

  103. Dave VK, Vickers CFH. Azathioprine in the treatment of mucocutaneous pemphigoid. Br J Dermatol 1974; 90: 183–6

    Article  PubMed  CAS  Google Scholar 

  104. Kirtschig G, Murrell D, Wojnarowska F, et al. Interventions for mucous membrane pemphigoid and epidermolysis bullosa acquisita. Cochrane Database Syst Rev 2003; (1): CD004056

    Google Scholar 

  105. Foster CS, Ahmed AR. Intravenous immunoglobulin therapy for ocular cicatricial pemphigoid. Ophthalmology 1999; 106:2136–43

    Article  PubMed  CAS  Google Scholar 

  106. Ahmed AR, Colon JE. Comparison between intravenous immunoglobulin and conventional immunosuppressive therapy regimens in patients with severe oral pemphigoid. Arch Dermatol 2001; 137: 1181–9

    PubMed  CAS  Google Scholar 

  107. Sami N, Bhol KC, Ahmed AR. Intravenous immunoglobulin therapy in patients with multiple mucosal involvement in mucous membrane pemphigoid. Clin Immunol Immunopathol 2002; 102: 59–67

    CAS  Google Scholar 

  108. Sami N, Bhol KC, Ahmed AR. Treatment of oral pemphigoid with intravenous immunoglobulin as mono-therapy. Long-term follow-up: influence of treatment on antibody titres to human α6 integrin. Clin Exp Immunol 2002; 129: 533–40

    Article  PubMed  CAS  Google Scholar 

  109. Mackay IR, Rosen FS. Immunomodulation of autoimmune and inflammatory diseases with intravenous immune globulin. N Engl J Med 2001; 345: 747–55

    Article  Google Scholar 

  110. Letko E, Bhol K, Foster CS, et al. Influence of intravenous immunoglobulin therapy on serum levels of anti-β4 antibodies in ocular cicatricial pemphigoid. Curr Eye Res 2000; 21: 646–54

    Article  PubMed  CAS  Google Scholar 

  111. Taverna JA, Lerner A, Bhawan J, et al. Successful adjuvant treatment of recalcitrant mucous membrane pemphigoid with anti-CD20 antibody rituximab. J Drugs Dermatol 2007 Jul; 6(7): 731–2

    PubMed  Google Scholar 

  112. Meyer-ter-Vehn T, Schmidt E, Zillikens D, et al. Mucous membrane pemphigoid with ocular involvement. Part II: therapy. Ophthalmologe 2008 Apr; 105(4): 405–19

    Article  PubMed  CAS  Google Scholar 

  113. Yaoita H, Briggaman RA, Lawley TJ, et al. Epidermolysis bullosa acquisita: ultrastructural and immunological studies. J Invest Dermatol 1981; 76: 288–92

    Article  PubMed  CAS  Google Scholar 

  114. Nieboer C, Boorsma DM, Woerdeman MJ, et al. Epidermolysis bullosa acquisita: immunofluorescence, electron microscopic and immunoelectron microscopic studies in four patients. Br J Dermatol 1980; 102: 383–92

    Article  PubMed  CAS  Google Scholar 

  115. Woodley DT, Briggaman RA, O’Keefe EJ, et al. Identification of the skin basement membrane autoantigen in epidermolysis bullosa acquisita. N Engl J Med 1984; 310: 1007–13

    Article  PubMed  CAS  Google Scholar 

  116. Sakai LY, Keene DR, Morris NP, et al. Type VII collagen is a major structural component of anchoring fibrils. J Cell Biol 1986; 103: 1577–86

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  118. Vodegel RM, de Jong MC, Pas HH, et al. IgA-mediated epidermolysis bullosa acquisita: two cases and review of the literature. J Am Acad Dermatol 2002 Dec; 47(6): 919–25

    Article  PubMed  Google Scholar 

  119. Engineer L, Ahmed AR. Emerging treatment for epidermolysis bullosa acquisita. J Am Acad Dermatol 2001; 44: 818–28

    Article  PubMed  CAS  Google Scholar 

  120. Cunningham BB, Kirchmann TTT, Woodley D. Colchicine for epidermolysis bullosa acquisita. J Am Acad Dermatol 1996; 34: 781–4

    Article  PubMed  CAS  Google Scholar 

  121. Megahed M, Scharffetter-Kochanek K. Epidermolysis bullosa acquisita: successful treatment with colchicine. Arch Dermatol Res 1994; 286: 35–40

    Article  PubMed  CAS  Google Scholar 

  122. Kofler H, Wambacher-Gasser B, Topar G, et al. Intravenous immunoglobulin treatment in therapy-resistant epidermolysis bullosa acquisita. J Am Acad Dermatol 1996; 34: 331–5

    Google Scholar 

  123. Gordon KB, Chan LS, Woodley DT. Treatment of refractory epidermolysis bullosa acquisita with extra-corporeal photochemotherapy. Br J Dermatol 1997; 136: 415–20

    Article  PubMed  CAS  Google Scholar 

  124. Heilborn JD, Stahle-Backdahl M, Albertioni F, et al. Low-dose oral pulse methotrexate as monotherapy in elderly patients with bullous pemphigoid. J Am Acad Dermatol 1999; 40: 741–8

    Article  PubMed  CAS  Google Scholar 

  125. Schmidt E, Benoit S, Brocker EB, et al. Successful adjuvant treatment of recalcitrant epidermolysis bullosa acquisita with anti-CD20 antibody rituximab. Arch Dermatol 2006 Feb; 142(2): 147–50

    Article  PubMed  Google Scholar 

  126. Ansel J, Petrozzi JW, Kumar V. Possible drug-induced pemphigus-like antibodies with clinical manifestation of erythema multiforme. Arch Dermatol 1983; 119: 1006–9

    Article  PubMed  CAS  Google Scholar 

  127. Coulson IH, Cook MG, Bruton J, et al. Atypical pemphigus vulgaris associated with angio-follicular lymph node hyperplasia (Castleman’s disease). Clin Exp Dermatol 1986; 11:656–63

    Article  PubMed  CAS  Google Scholar 

  128. Gili A, Ngan BY, Lester R. Castleman’s disease associated with pemphigus vulgaris. J Am Acad Dermatol 1992; 25: 955–9

    Article  Google Scholar 

  129. Tagami S, Imamura S, Noguchi S, et al. Co-existence of peculiar pemphigus, myasthenia gravis and malignant thymoma. Dermatologica 1976; 152: 181–90

    Article  PubMed  CAS  Google Scholar 

  130. Anhalt GJ, Kim S, Stanley JR, et al. Paraneoplastic pemphigus: an autoimmune mucocutaneous disease associated with neoplasia. N Engl J Med 1990; 323: 1729–35

    Article  PubMed  CAS  Google Scholar 

  131. Fullerton SH, Woodley DT, Smoller BR, et al. Paraneoplastic pemphigus with autoantibody deposition in bronchial epithelium after autologous bone marrow transplantation. JAMA 1992; 267: 1500–2

    Article  PubMed  CAS  Google Scholar 

  132. Lam S, Stone MS, Goeken JA, et al. Paraneoplastic pemphigus, cicatricial conjunctivitis, and acanthosis nigricans with pachydermatoglyphy in a patient with bronchogenic squamous cell carcinoma. Ophthalmology 1992; 99(1): 108–13

    PubMed  CAS  Google Scholar 

  133. Anhalt GJ. Paraneoplastic pemphigus. Adv Dermatol 1997; 12: 77–96; discussion 97

    PubMed  CAS  Google Scholar 

  134. Camisa C, Helm TN, Liu YC, et al. Paraneoplastic pemphigus: a report of three cases including one long-term survivor. J Am Acad Dermatol 1992 Oct; 27(4): 547–53

    Article  PubMed  CAS  Google Scholar 

  135. Hertzberg MS, Schifter M, Sullivan J, et al. Paraneoplastic pemphigus in two patients with B cell non-Hodgkin’s lymphoma: significant responses to cyclophosphamide and prednisolone. Am J Hematol 2000; 63: 105–6

    Google Scholar 

  136. Williams JV, Marks Jr JG, Billingsley EM. Use of mycophenolate mofetil in the treatment of paraneoplastic pemphigus. Br J Dermatol 2000; 142: 506–8

    Article  PubMed  CAS  Google Scholar 

  137. Dega H, LaPorte JL, Joly P, et al. Paraneoplastic pemphigus associated with Hodgkin’s disease. Br J Dermatol 1998; 138: 196–7

    Article  PubMed  CAS  Google Scholar 

  138. Robinson ND, Hashimoto T, Amagai M, et al. The new pemphigus variants. J Am Acad Dermatol 1999; 40: 649–71

    Article  PubMed  CAS  Google Scholar 

  139. Korman NJ. New and emerging therapies in the treatment of blistering diseases. Dermatol Clin 2000; 18: 127–37

    Article  PubMed  CAS  Google Scholar 

  140. Krunic ALJ, Kokai D, Bacetic B, et al. Retroperitoneal round-cell liposarcoma associated with paraneoplastic pemphigus presenting as lichen planus pemphigoides-like eruption. Int J Dermatol 1997; 36: 526–9

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgements

No sources of funding were used to assist in the preparation of this review. The author has no conflicts of interest that are directly relevant to the content of this review.

Author information

Authors and Affiliations

  1. Department of Dermatology, University of Cincinnati, College of Medicine, 231 Albert Sabin Way, P.O. Box 670592, Cincinnati, Ohio, 45267-0592, USA

    Diya F. Mutasim

Authors
  1. Diya F. Mutasim
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Diya F. Mutasim.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Mutasim, D.F. Autoimmune Bullous Dermatoses in the Elderly. Drugs Aging 27, 1–19 (2010). https://doi.org/10.2165/11318600-000000000-00000

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.2165/11318600-000000000-00000

Keywords

Search

Navigation