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Vesiculobullous Dermatoses

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Buka's Emergencies in Dermatology

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Abstract

The vesicobullous diseases in dermatology are numerous and quite distinct from one another; the unifying feature, intuitively, is the histologic feature of separation either intraepidermally or subepidermally. Vesicobullous diseases can be mediated through infections, allergic causes, physical insults, or may occur as a result of autoimmune and hereditary disorders. The majority of vesicobullous diseases are acquired, wherein autoantibodies target specific cellular adhesion molecules resulting in separation of keratinocytes. In contrast, inherited vesicobullous diseases due to genetic loss of either adhesion molecules or basic structural proteins, lead to loss of cell–cell adhesion following minor or insignificant trauma or traction to the skin. The term “acantholysis” refers to intraepidermal pathology, whereas “blister” is traditionally used to delineate subepidermal disease. Whether acquired or inherited, vesicobullous diseases are a major source of patient morbidity and mortality in dermatology, either due to inherent disease processes or from therapeutic complications.

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References

  1. Powell AM, Black M. A stepwise approach to the diagnosis of blisters in the clinic. Clin Dermatol. 2001;19:598–606.

    Article  PubMed  CAS  Google Scholar 

  2. Harman KE. New laboratory techniques for the assessment of acquired immunobullous disorders. Clin Exp Dermatol. 2002;27:40–6.

    Article  PubMed  CAS  Google Scholar 

  3. Mihai S, Sitaru C. Immunopathology and molecular diagnosis of autoimmune bullous diseases. J Cell Mol Med. 2007;11:462–81.

    Article  PubMed  CAS  Google Scholar 

  4. Fine JD. Management of acquired bullous skin diseases. N Engl J Med. 1995;30:1475–84.

    Google Scholar 

  5. Browne BJ, Edwards B, Rogers RL. Dermatologic emergencies. Prim Care Clin Office Pract. 2006;33:685–95.

    Article  Google Scholar 

  6. Fuertes I, Guilabert A, Mascaro JM, et al. Rituximab in childhood pemphigus vulgaris: a long-term follow-up case and review of the literature. Dermatology. 2010;221:13–6.

    Article  PubMed  Google Scholar 

  7. Yeh SW, Sami N, Ahmed RA. Treatment of pemphigus vulgaris: current and emerging options. Am J Clin Dermatol. 2005;6(5):327–42.

    Article  PubMed  Google Scholar 

  8. Black M, Mignogna MD, Scully C. Number II. Pemphigus vulgaris. Oral Dis. 2005;11(3):119–30.

    Article  PubMed  CAS  Google Scholar 

  9. Bastuji-Garin S, Souissi R, Blum L, et al. Comparative epidemiology of pemphigus in Tunisia and France: unusual incidence of pemphigus foliaceus in young Tunisian women. J Invest Dermatol. 1995;104:302–5.

    Article  PubMed  CAS  Google Scholar 

  10. Firooz A, Mazhar A, Ahmed AR. Prevalence of autoimmune diseases in the family members of patients with pemphigus vulgaris. J Am Acad Dermatol. 1994;31(3):434–7.

    Article  PubMed  CAS  Google Scholar 

  11. Ahmed AR, Wagner R, Khatri K, et al. Major histocompatibility complex haplotypes and class II genes in non-Jewish patients with pemphigus vulgaris. Proc Natl Acad Sci U S A. 1991;88(11):5056–60.

    Article  PubMed  CAS  Google Scholar 

  12. Lombardi ML, Mercuro O, Ruocco V, et al. Common human leukocyte antigen alleles in pemphigus vulgaris and pemphigus foliaceus Italian patients. J Invest Dermatol. 1999;113(1):107–10.

    Article  PubMed  CAS  Google Scholar 

  13. Szafer F, Brautbar C, Tzfoni E, et al. Detection of disease-specific restriction fragment length polymorphisms in pemphigus vulgaris linked to the DQw1 and DQw3 alleles of the HLA-D region. Proc Natl Acad Sci U S A. 1987;84(18):6542–5.

    Article  PubMed  CAS  Google Scholar 

  14. Matzner Y, Erlich HA, Brautbar C, et al. Identical HLA class II alleles predispose to drug-triggered and idiopathic pemphigus vulgaris. Acta Derm Venereol. 1995;75(1):12–4.

    PubMed  CAS  Google Scholar 

  15. Sinha AA, Brautbar C, Szafer F, et al. A newly characterized HLA DQ beta allele associated with pemphigus vulgaris. Science. 1988;239(4843):1026–9.

    Article  PubMed  CAS  Google Scholar 

  16. Fedeles F, Murphy M, Rothe MJ, et al. Nutrition and bullous skin diseases. Clin Dermatol. 2010;28:627–43.

    Article  PubMed  Google Scholar 

  17. Ruocco V, Brenner S, Lombardi ML. A case of diet-related pemphigus. Dermatology. 1996;192:373–4.

    Article  PubMed  CAS  Google Scholar 

  18. Chorzelski TPHT, Jablonska S, et al. Can pemphigus vulgaris be induced by nutritional factors? Eur J Dermatol. 1996;6:284–6.

    Google Scholar 

  19. Lee AN, Werth VP. Activation of autoimmunity following use of immunostimulatory herbal supplements. Arch Dermatol. 2004;140:723–7.

    Article  PubMed  Google Scholar 

  20. Brenner S, Goldberg I. Drug-induced pemphigus. Clin Dermatol. 2011;29(4):455–7.

    Article  PubMed  Google Scholar 

  21. Bhol K, Mohimen A, Ahmed AR. Correlation of subclasses of IgG with disease activity in pemphigus vulgaris. Dermatology. 1994;189 Suppl 1:85–9.

    Article  PubMed  Google Scholar 

  22. Wilson CL, Wojnarowska F, Dean D, Pasricha JS. IgG subclasses in pemphigus in Indian and UK populations. Clin Exp Dermatol. 1993;18(3):226–30.

    Article  PubMed  CAS  Google Scholar 

  23. Amagai M, Tsunoda K, Zillikens D, et al. The clinical phenotype of pemphigus is defined by the anti-desmoglein autoantibody profile. J Am Acad Dermatol. 1999;40:167–70.

    Article  PubMed  CAS  Google Scholar 

  24. Keehn CA, Morgan MB. Clinicopathologic attributes of common geriatric dermatologic entities. Dermatol Clin. 2004;22(1):115–23 [vii.].

    Article  PubMed  Google Scholar 

  25. Parker SRS, MacKelfresh J. Autoimmune blistering diseases in the elderly. Clin Dermatol. 2011;29:69–79.

    Article  PubMed  Google Scholar 

  26. Knudson RM, Kalaaji AN, Bruce AJ. The management of mucous membrane pemphigoid and pemphigus. Dermatol Ther. 2010;23:268–80.

    Article  PubMed  Google Scholar 

  27. Mahoney MG, Wang Z, Rothenberger K, et al. Explanations for the clinical and microscopic localization of lesions in pemphigus foliaceus and vulgaris. J Clin Invest. 1999;103:461–8.

    Article  PubMed  CAS  Google Scholar 

  28. Ide A, Hashimoto T, Amagai M, et al. Detection of autoantibodies against bullous pemphigoid and pemphigus antigens by an enzyme-linked immunosorbent assay using the bacterial recombinant proteins. Exp Dermatol. 1995;4:112–6.

    Article  PubMed  CAS  Google Scholar 

  29. Ishii K, Amagai M, et al. Detection of autoantibodies against bullous pemphigoid and pemphigus antigens by an enzyme-linked immunosorbent assay using the bacterial recombinant proteins. Exp Dermatol. 1995;4:112–6.

    Article  Google Scholar 

  30. Risser J, Lewis K. Weinstock MA Mortality of bullous skin disorders from 1979 through 2002 in the United States. Arch Dermatol. 2009;145(9):1005–8.

    Article  PubMed  Google Scholar 

  31. Seidenbaum M, David M, Sandbank M. The course and prognosis of pemphigus. A review of 115 patients. Int J Dermatol. 1988;27:580–4.

    Article  PubMed  CAS  Google Scholar 

  32. Bystryn JC, Steinman NM. The adjuvant therapy of pemphigus. An update. Arch Dermatol. 1996;132:203–12.

    Article  PubMed  CAS  Google Scholar 

  33. Fleming TE, Korman NJ. Cicatricial pemphigoid. J Am Acad Dermatol. 2000;43:571–91.

    Article  PubMed  CAS  Google Scholar 

  34. Lee MS, Wakefield PE, Lonzelman JL, James WD. Oral insertable prosthetic device as an aid in treating oral ulcers. Arch Dermatol. 1991;127:479–80.

    Article  PubMed  CAS  Google Scholar 

  35. Ahmed AR, Kurgis BS, Rogers RS. Cicatricial pemphigoid. J Am Acad Dermatol. 1991;24:987–1001.

    Article  PubMed  CAS  Google Scholar 

  36. Muzyka BC, Greenberg MS, Glick MG. Management of oral manifestations of mucous membrane pemphigoid. J Geriatr Dermatol. 1994;2:105–9.

    Google Scholar 

  37. Amagai M, Komai A, Hashimoto T, et al. Usefulness of enzyme-linked immunosorbent assay (ELISA) using recombinant esmogleins 1 and 3 for serodiagnosis of pemphigus. Br J Dermatol. 1999;140:351–7.

    Article  PubMed  CAS  Google Scholar 

  38. Khan YK, Kalaaji AN, Clarke BL. Prevention and treatment of glucocorticoid-induced osteoporosis in dermatologic practice: a review. J Drugs Dermatol. 2008;7:2–8.

    Google Scholar 

  39. Toth GG, Jonkman MF. Therapy of pemphigus. Clin Dermatol. 2001;19:761–7.

    Article  PubMed  CAS  Google Scholar 

  40. Gerhart J, Kalaaji A. Development of Pneumocystis carinii pneumonia in patients with immunobullous and connective tissue disease receiving immunosuppressive medications. J Am Acad Dermatol. 2010;62(6):957–61.

    Article  PubMed  CAS  Google Scholar 

  41. Lehman JS, Kalaaji AN. Role of primary prophylaxis for pneumocystis pneumonia in patients treated with systemic corticosteroids or other immunosuppressive agents for immune-mediated dermatologic conditions. J Am Acad Dermatol. 2010;63(5):815–23.

    Article  PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  43. Hornsten P, Keisu M, Wiholm B. The incidence of agranulocytosis during treatment of dermatitis herpetiformis with dapsone as reported in Sweden, 1972 through 1988. Arch Dermatol. 1990;126:919–22.

    Article  PubMed  CAS  Google Scholar 

  44. Raizman MB, Fay AM, Weiss JS. Dapsone-induced neutropenia in patients treated for ocular cicatricial pemphigoid. Ophthalmology. 1994;101:1805–7.

    PubMed  CAS  Google Scholar 

  45. Aberer W, Wolff SE, Stingl G, Wolff K. Azathioprine in the treatment of pemphigus vulgaris. A long-term follow-up. J Am Acad Dermatol. 1987;16:527–33.

    Article  PubMed  CAS  Google Scholar 

  46. Roenigk HH, Deodar S. Pemphigus treatment with azathioprine. Arch Dermatol. 1973;107:353–7.

    Article  PubMed  Google Scholar 

  47. Nousari HC, Anhalt GJ. The role of mycophenolate mofetil in the management of pemphigus. J Am Acad Dermatol. 1999;135:853–4.

    CAS  Google Scholar 

  48. Beissert S, Werfel T, Frieling U, et al. A comparison of oral methylprednisolone plus azathioprine or mycophenolate mofetil for the treatment of pemphigus. Arch Dermatol. 2006;142:1447–54.

    Article  PubMed  CAS  Google Scholar 

  49. Kirtschig G, Khumalo NP. Management of bullous pemphigoid: recommendations for immunomodulatory treatments. Am J Clin Dermatol. 2004;5:319–26.

    Article  PubMed  Google Scholar 

  50. Fellner MJ, Katz JM, McCabe JB. Successful use of cyclophosphamide and prednisone for initial treatment of pemphigus vulgaris. Arch Dermatol. 1978;114:889–94.

    Article  PubMed  CAS  Google Scholar 

  51. Harman KE, Albert S, Black MM. Guidelines for the management of pemphigus vulgaris. Br J Dermatol. 2003;149:926–36.

    Article  PubMed  CAS  Google Scholar 

  52. Ioannides D, Chrysomallis F, Bystryn JC. Ineffectiveness of cyclosporine as an adjuvant to corticosteroids in the treatment of pemphigus. Arch Dermatol. 2000;136:868–72.

    Article  PubMed  CAS  Google Scholar 

  53. El Tal AK, Posner MR, Spigelman Z, Ahmed AR. Rituximab: a monoclonal antibody to CD20 used in the treatment of pemphigus vulgaris. J Am Acad Dermatol. 2006;55:449–59.

    Article  PubMed  Google Scholar 

  54. Niedermeier A. Delayed response of oral pemphigus vulgaris to rituximab treatment. Eur J Dermatol. 2006;16:266–70.

    PubMed  Google Scholar 

  55. Pandya AG, Dyke C. Treatment of pemphigus with gold. Arch Dermatol. 1998;134:1104–7.

    Article  PubMed  CAS  Google Scholar 

  56. Penneys NS, Eaglstein WH, Frost P. Management of pemphigus with gold compounds. A long-term follow-up report. Arch Dermatol. 1976;112:185–7.

    Article  PubMed  CAS  Google Scholar 

  57. Jolles S. A review of high-dose intravenous immunoglobulin (hdIVIg) in the treatment of the autoimmune blistering disorders. Clin Exp Dermatol. 2001;26:127–31.

    Article  PubMed  CAS  Google Scholar 

  58. Lee F. Longterm clinical remission of oral and cutaneous pemphigus with thalidomide. Int J Dermatol. 2011;50:1120–3.

    Article  PubMed  Google Scholar 

  59. Ahmed AR, Blose DA. Pemphigus vegetans. Neumann type and Hallopeau type. Int J Dermatol. 1984;23:135–41.

    Article  PubMed  CAS  Google Scholar 

  60. Becker BA, Gaspari AA. Pemphigus vulgaris and vegetans. Dermatol Clin. 1993;11:429–52.

    PubMed  CAS  Google Scholar 

  61. Joly P, Litrowski N. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clin Dermatol. 2011;29:432–6.

    Article  PubMed  Google Scholar 

  62. James KA, Culton DA, Diaz LA. Diagnosis and clinical features of pemphigus foliaceus. Dermatol Clin. 2011;29(3):405–12.

    Article  PubMed  CAS  Google Scholar 

  63. Kraigher O, Wohl Y, Gat A, Brenner S. A mixed immunoblistering disorder exhibiting features of bullous pemphigoid and pemphigus foliaceus associated with Spirulina algae intake. Int J Dermatol. 2008;47:61–3.

    Article  PubMed  Google Scholar 

  64. Steffen C, Thomas D. The men behind the eponym: Francis E. Senear, Barney Usher, and the Senear-Usher syndrome. Am J Dermatopathol. 2003;25(5):432–6.

    Article  PubMed  Google Scholar 

  65. Schmidt E. Zillikens D Modern diagnosis of autoimmune blistering skin diseases. Autoimmun Rev. 2010;10:84–9.

    Article  PubMed  CAS  Google Scholar 

  66. Grando SA. New approaches to the treatment of pemphigus. J Investig Dermatol Symp Proc. 2004;9(1):84–91.

    Article  PubMed  Google Scholar 

  67. Gupta R. Prolonged remission of pemphigus induced by dexamethasone-cyclophosphamide pulse therapy. Indian J Dermatol Venereol Leprol. 2007;73(2):121–2.

    Article  PubMed  Google Scholar 

  68. Katz KH, Marks Jr JG, Helm KF. Pemphigus foliaceus successfully treated with mycophenolate mofetil as a steroid-sparing agent. J Am Acad Dermatol. 2000;42(3):514–5.

    Article  PubMed  CAS  Google Scholar 

  69. Dumas V, Roujeau JC, Wolkenstein P, Revuz J, Cosnes A. The treatment of mild pemphigus vulgaris and pemphigus foliaceus with a topical corticosteroid. Br J Dermatol. 1999;140(6):1127–9.

    Article  PubMed  CAS  Google Scholar 

  70. Cianchi G, Lembo L, Colonna L, et al. Pemphigus foliaceus induced by radiotherapy and responsive to dapsone. J Dermatolog Treat. 2006;17(4):244–6.

    Article  Google Scholar 

  71. Friedman H, Campbell I, Rocha-Alvarez R, et al. Endemic pemphigus foliaceus (fogo selvagem) in native Americans from Brazil. J Am Acad Dermatol. 1995;32:949–56.

    Article  PubMed  CAS  Google Scholar 

  72. Warren SJ, Lin MS, Giudice GJ, et al. The prevalence of antibodies against desmoglein 1 in endemic pemphigus foliaceus in Brazil. Cooperative Group on Fogo Selvagem Research. N Engl J Med. 2000;343(1):23–30.

    Article  PubMed  CAS  Google Scholar 

  73. Li N, Aoki V, Hans-Filho G, et al. The role of intramolecular epitope spreading in the pathogenesis of endemic pemphigus foliaceus (fogo selvagem). J Exp Med. 2003;197:1501–10.

    Article  PubMed  CAS  Google Scholar 

  74. Ribeiro JM, Valenzuela JG, Pham VM, et al. An insight into the sialotranscriptome of Simulium nigrimanum, a black fly associated with fogo selvagem in South America. Am J Trop Med Hyg. 2010;82:1060–75.

    Article  PubMed  CAS  Google Scholar 

  75. Tur E, Brenner S, Lombardi ML. A case of diet-related pemphigus. Dermatology. 1996;192:373–4.

    Article  Google Scholar 

  76. 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 

  77. Kimyal-Asadi A. Jih MH Paraneoplastic pemphigus. Int J Dermatol. 2001;40(6):367–72.

    Article  Google Scholar 

  78. Amgai M, Nishikawa T, Nousari HC, et al. Antibodies against desmoglein 3 (pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice. J Clin Invest. 1998;102:775–82.

    Article  Google Scholar 

  79. Anhalt GJ. Paraneoplastic pemphigus. J Invest Dermatol Symp Proc. 2004;9:29–33.

    Article  Google Scholar 

  80. Fine JD, Eady RAJ, Bauer EA, et al. Revised classification system for inherited epidermolysis bullosa: report of the Second International Consensus Meeting on diagnosis and classification of epidermolysis bullosa. J Am Acad Dermatol. 2000;42:1051–66.

    Article  PubMed  CAS  Google Scholar 

  81. Fuchs E, Coulombe P, Cheng J, et al. Genetic bases of epidermolysis bullosa simplex and epidermolytic hyperkeratosis. J Invest Dermatol. 1994;103(S):25–30.

    Article  Google Scholar 

  82. Haynes L. Nutritional support for children with epidermolysis bullosa. Br J Nurs. 2006;15:1097–101.

    PubMed  Google Scholar 

  83. Gruskay DM. Nutritional management in the child with epidermolysis bullosa. Arch Dermatol. 1988;124:760–1.

    Article  PubMed  CAS  Google Scholar 

  84. Fine JD, Johnson LB, Weiner M, et al. Gastrointestinal complications of inherited epidermolysis bullosa: cumulative experience of the National Epidermolysis Bullosa Registry. J Pediatr Gastroenterol Nutr. 2008;46:147–58.

    Article  PubMed  Google Scholar 

  85. Gamelli RL. Nutritional problems of the acute and chronic burn patient. Relevance to epidermolysis bullosa. Arch Dermatol. 1988;124:756–9.

    Article  PubMed  CAS  Google Scholar 

  86. Retief CR, Malkinson FD, Pearson RW. Two familial cases of epidermolysis bullosa simplex successfully treated with tetracycline. Arch Dermatol. 1999;135:997–8.

    Article  PubMed  CAS  Google Scholar 

  87. Fine JD, Eady RAJ. Tetracycline and epidermolysis bullosa simplex-a new indication for one of the oldest and most widely used drugs in dermatology. Arch Dermatol. 1999;135:981–2.

    Article  PubMed  CAS  Google Scholar 

  88. Schmidt E, Zillikens D. Autoimmune and inherited subepidermal blistering diseases: advances in the clinic and the laboratory. Adv Dermatol. 2000;16:113–57.

    PubMed  CAS  Google Scholar 

  89. Hall WH, Ramachandran R, Narayan S, et al. An electronic application for rapidly calculating Charlson comorbidity score. BMC Cancer. 2004;4:94.

    Article  PubMed  Google Scholar 

  90. Jassal SV, Chiu E, Hladuewich M. Loss of independence of patients starting dialysis at 80 years of age or older. N Engl J Med. 2009;361:1612–3.

    Article  PubMed  CAS  Google Scholar 

  91. Posner BM, Jette AM, Smith KW, et al. Nutrition and health risks in the elderly: the nutrition screening initiative. Am J Public Health. 1993;83:972–8.

    Article  PubMed  CAS  Google Scholar 

  92. Marazza G, Pham HC, Scharer L, et al. Incidence of bullous pemphigoid and pemphigus in Switzerland: a 2-year prospective study. Br J Dermatol. 2009;161:861–8.

    Article  PubMed  CAS  Google Scholar 

  93. Delgado JC, Turbay D, Yunis EJ, et al. A common major histocompatibility complex class II allele HLA-DQB1*0301 is present in clinical variants of pemphigoid. Proc Natl Acad Sci USA. 1996;93:8569–71.

    Article  PubMed  CAS  Google Scholar 

  94. Nozu T, Mita H. Bullous pemphigoid and percutaneous endoscopic gastrostomy. Intern Med. 2010;49:971–5.

    Article  PubMed  Google Scholar 

  95. Sheerin N, Bourke JF, Holder J, North J, Burns DA. Bullous pemphigoid following radiotherapy. Clin Exp Dermatol. 1995;20:80–2.

    Article  PubMed  CAS  Google Scholar 

  96. Balato N, Ayala F, Patruno C, Turco P, Ruocco V. Bullous pemphigoid induced by a thermal burn. Int J Dermatol. 1994;33:55–6.

    Article  PubMed  CAS  Google Scholar 

  97. Parslew R, Verbov JL. Bullous pemphigoid at sites of trauma. Br J Dermatol. 1997;137:825–6.

    Article  PubMed  CAS  Google Scholar 

  98. Khandpur S, Verma P. Bullous pemphigoid. Indian J Dermatol Venereol Leprol. 2011;77(4):450–5.

    Article  PubMed  Google Scholar 

  99. Barnadas M, Rubiales V, Gonzalez J, et al. Enzyme-linked immunosorbent assay (ELISA) and indirect immunofluorescence testing in a bullous pemphigoid and pemphigoid gestationis. Int J Dermatol. 2008;47:1245–9.

    Article  PubMed  Google Scholar 

  100. Joly P, Roujeau JC, Benichou J, et al. A comparison of oral and topical corticosteroids in the treatment of patients with bullous pemphigoid: a multicenter randomized study. J Invest Dermatol. 2009;129:1681–7.

    Article  PubMed  CAS  Google Scholar 

  101. Egan CA, Lazarova Z, Darling TN, et al. Anti-epiligrin cicatricial pemphigoid and relative risk for cancer. Lancet. 2001;357:1850–1.

    Article  PubMed  CAS  Google Scholar 

  102. Roth MM. Pregnancy dermatoses, diagnosis management and controversies. Am J Clin Dermatol. 2011;12(1):25–41.

    Article  PubMed  Google Scholar 

  103. Djahansouzi S, Nestle-Kraemling C, Dall P, et al. Herpes gestationis may present itself as a paraneoplastic syndrome of choriocarcinoma-a case report. Gynecol Oncol. 2003;89(2):334–7.

    Article  PubMed  CAS  Google Scholar 

  104. do Valle Chiossi MP, Costa RS, Ferreira Roselino AM. Titration of herpes gestationis factor fixing to C3 in pemphigoid herpes gestationis associated with choriocarcinoma. Arch Dermatol. 2000; 136(1):129-30.

    Google Scholar 

  105. Yancey KB. Herpes gestationis. Dermatol Clin. 1990;8:727–34.

    PubMed  CAS  Google Scholar 

  106. Jenkins RE, Hern S, Black MM. Clinical features and management of 87 patients with pemphigoid gestationis. Clin Exp Dermatol. 1999;24:255–9.

    Article  PubMed  CAS  Google Scholar 

  107. Paunescu MM, Feier V, Paunescu M. Dermatoses of pregnancy. Acta Dermatovenerol Alp Panonica Adriat. 2008;17(1):4–11.

    PubMed  Google Scholar 

  108. Shornick JK. Herpes gestationis. J Am Acad Dermatol. 1987;17:539–56.

    Article  PubMed  CAS  Google Scholar 

  109. Yunis JJ, Mobini N, Yunis EJ, et al. Common major histocompatibility complex class II markers in clinical variants of cicatricial pemphigoid. Proc Natl Acad Sci U S A. 1994;91:7747–51.

    Article  PubMed  CAS  Google Scholar 

  110. Chan LS, Ahmed AR, Anhalt GF, et al. The first international consensus on mucous membrane pemphigoid. Arch Dermatol. 2002;43:571–91.

    Google Scholar 

  111. Foster CS. Cicatricial pemphigoid. Trans Am Ophthalmol Soc. 1986;85:527–663.

    Google Scholar 

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

    PubMed  CAS  Google Scholar 

  113. Ahmed AR, Dahl MV. Consensus statement on the use of intravenous immunoglobulin therapy in the treatment of autoimmune mucocutaneous blistering diseases. Arch Dermatol. 2003;139:1051–9.

    Article  PubMed  CAS  Google Scholar 

  114. Canizares MJ, Smith DI, Conners MS, et al. Successful treatment of mucous membrane pemphigoid with etanercept in 3 patients. Arch Dermatol. 2006;142:1457–61.

    Article  PubMed  CAS  Google Scholar 

  115. Sacher C, Rubbert A, Konig C, et al. Treatment of recalcitrant cicatricial pemphigoid with the tumor necrosis factor alpha antagonist etanercept. J Am Acad Dermatol. 2022;46:113–5.

    Article  Google Scholar 

  116. Hefferman MP, Bentley DD. Successful treatment of mucous membrane pemphigoid with infliximab. Arch Dermatol. 2006;142:1268–70.

    Article  Google Scholar 

  117. Ross A, Jaycock P, Cook S, et al. The use of rituximab in refractory mucous membrane pemphigoid with severe ocular involvement. Br J Ophthalmol. 2009;93:421–2.

    Article  PubMed  CAS  Google Scholar 

  118. Schumann T, Schmidt E, Booken N, et al. Successful treatment of mucous membrane pemphigoid with the anti-CD20 antibody rituximab. Acta Derm Venereol. 2009;89:101–2.

    PubMed  Google Scholar 

  119. 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;6:731–2.

    PubMed  Google Scholar 

  120. Duong DJ, Moxley RT, Kellman RM, et al. Thalidomide therapy for cicatricial pemphigoid. J Am Acad Dermatol. 2002;47:S193–5.

    Article  PubMed  Google Scholar 

  121. Donnenfelt ED, Perry HD, Wallerstein A, et al. Subconjunctival mitomycin C for the treatment of ocular cicatricial pemphigoid. Ophthalmology. 1999;106:72–8.

    Article  Google Scholar 

  122. Wojnarowska F, Marsden RA, Bhogal B, Black MM. Chronic bullous disease of childhood, childhood cicatricial pemphigoid, and linear IgA disease of adults. A comparative study demonstrating clinical and immunopathologic overlap. J Am Acad Dermatol. 1988;19(5):792–805.

    Article  PubMed  CAS  Google Scholar 

  123. Esterly NB, Furey NL, Kirschner BS, et al. Chronic bullous dermatosis of childhood. Arch Dermatol. 1977;113:42–6.

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  125. Wojnarowska F, Marsden RA, Bhogal B, et al. Childhood cicatricial pemphigoid with linear IgA deposits. Clin Exp Dermatol. 1984;9:407–15.

    Article  PubMed  CAS  Google Scholar 

  126. Kuechle MK, Stegemeir E, Maynard B, et al. Drug-induced linear IgA bullous dermatosis: report of six cases and review of the literature. J Am Acad Dermatol. 1994;30:187–92.

    Article  PubMed  CAS  Google Scholar 

  127. Mobacken H, Kastrup W, Ljundhall K, et al. Linear IgA dermatosis: a study of ten adult patients. Acta Derm Venereol. 1983;63:123–8.

    PubMed  CAS  Google Scholar 

  128. Dabrowski J, Chorzelski TP, Jablonska S, et al. Immunoelectron microscopic studies in IgA linear dermatosis. Arch Dermatol Res. 1979;265:289–98.

    Article  PubMed  CAS  Google Scholar 

  129. Chen M, Chan LS, Cai X, et al. Development of an ELISA for rapid detection of anti-type VII collagen autoantibodies in epidermolysis bullosa acquisita. J Invest Dermatol. 1997;108:68–72.

    Article  PubMed  CAS  Google Scholar 

  130. Leonard JN, Haffenden GP, Ring NP, et al. Linear IgA disease in adults. Br J Dermatol. 1982;107:301–16.

    Article  PubMed  CAS  Google Scholar 

  131. Wieme N, Lambert J, Moerman M, et al. Epidermolsis bullosa acquisita with combined features of bullous pemphigoid and cicatrical pemphigoid. Dermatology. 1999;198:310–3.

    Article  PubMed  CAS  Google Scholar 

  132. Zillikens D, Wever S, Roth A, et al. Incidence of autoimmune subepidermal blistering dermatoses in a region of central Germany. Arch Dermatol. 1995;131:957–8.

    Article  PubMed  CAS  Google Scholar 

  133. Nanda A, Dvorak R, Al-Saeed K, et al. Spectrum of autoimmune bullous diseases in Kuwait. Int J Dermatol. 2004;43:876–81.

    Article  PubMed  Google Scholar 

  134. Wong SN, Chua SH. Spectrum of subepidermal immunobullous disorders seen at the National Skin Centre Singapore: a 2-year review. Br J Dermatol. 2002;147:476–80.

    Article  PubMed  CAS  Google Scholar 

  135. Gammon WR. Epidermolysis bullosa acquisita. Semin Dermatol. 1988;7:218–24.

    PubMed  CAS  Google Scholar 

  136. Zumelzu C, Le Roux-Villet C, Loiseau P, et al. Black patients of African descent and HLA-DRB1*15:03 frequency overrepresented in epidermolysis bullosa acquisita. J Invest Dermatol. 2011;131:2386–93 [Epub ahead of print].

    Article  PubMed  CAS  Google Scholar 

  137. Hallel-Halevy D, Nadelman C, Chen M, et al. Epidermolysis bullosa acquisita update and review. Clin Dermatol. 2001;19:712–18.

    Article  PubMed  CAS  Google Scholar 

  138. 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 

  139. Gammon WR, Kowalwski C, Chorzelski TP, et al. Direct immunofluorescence studies of sodium ­chloride-separated skin in the differential diagnosis of bullous pemphigoid and epidermolysis bullosa aquisita. J Am Acad Dermatol. 1990;22:664–70.

    Article  PubMed  CAS  Google Scholar 

  140. Caux F, Kirtschig G, Lemarchand-Venencie F, et al. IgA epidermolysis bullosa aquisita in a child resulting in blindness. Br J Dermatol. 1997;137:270–5.

    Article  PubMed  CAS  Google Scholar 

  141. Fine JD, Bauer EA, McGuire J, Moshell A, editors. Epidermolysis bullosa: clinical, epidemiologic, and laboratory advances and the findings of the National Epidermolysis Bullosa Registry. Baltimore, MD: Johns Hopkins University Press; 1999.

    Google Scholar 

  142. Christiano AM, Uitto J. Molecular diagnosis of inherited skin diseases: the paradigm of dystrophic epidermolysis bullosa. Adv Dermatol. 1996;11:199–214.

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  144. Christiano AM, Fine JD, Uitto J. Genetic basis of dominantly inherited transient bullous dermolysis of the newborn: a splice site mutation in the type VII collagen gene. J Invest Dermatol. 1997;109:811–4.

    Article  PubMed  CAS  Google Scholar 

  145. Wright JT, Fine JD, Johnson LB. Hereditary epidermolysis bullosa: oral manifestations and dental management. Pediatr Dent. 1993;15:242–8.

    PubMed  CAS  Google Scholar 

  146. Wright JT, Fine JD, Johnson L. Dental caries risk in hereditary epidermolysis bullosa. Pediatr Dent. 1994;16:427–32.

    PubMed  CAS  Google Scholar 

  147. Fine JD, Johnson LB, Weiner M, et al. Pseudosyndactyly and musculoskeletal deformities in inherited epidermolysis bullosa (EB): experience of the National Epidermolysis Bullosa Registry, 1986-2002. J Hand Surg [Br]. 2005;30:14–22.

    Article  Google Scholar 

  148. Melville C, Atherton D, Burch M, et al. Fatal cardiomyopathy in dystrophic epidermolysis bullosa. Br J Dermatol. 1996;135:603–6.

    Article  PubMed  CAS  Google Scholar 

  149. Fine JD, Johnson LB, Weiner M, Suchindran C. Tracheolaryngeal complications of inherited epidermolysis bullosa (EB): experience of the National EB registry. Laryngoscope. 2007;117(9):1652–60.

    Article  PubMed  Google Scholar 

  150. Fine JD, Johnson LB, Suchindran C, et al. Cancer and inherited epidermolysis bullosa: lifetable analyses of the National Epidermolysis Bullosa Registry study population. In: Fine JD, Bauer EA, McGuire J, Moshell A, editors. Epidermolysis bullosa: clinical, epidemiologic, and laboratory advances, and the findings of the national epidermolysis bullosa registry. Baltimore, MD: Johns Hopkins University Press; 1999. p. 175–92.

    Google Scholar 

  151. Bauer EA, Cooper TW, Tucker DR, Esterly NB. Phenytoin therapy of recessive dystrophic epidermolysis bullosa. Clinical trial and prposed mechanism of action on collagenase. N Engl J Med. 1980;303:776–81.

    Article  PubMed  CAS  Google Scholar 

  152. Fine JD, Johnson L. Efficacy of systemic phenytoin in the treatment of junctional epidermolysis bullosa. Arch Dermatol. 1988;124:1402–6.

    Article  PubMed  CAS  Google Scholar 

  153. Fritsch P, Klein G, Aubock J, Hintner H. Retinoid therapy of recessive dystrophic epidermolysis bullosa [Letter]. J Am Acad Dermatol. 1983;9:766–7.

    Article  PubMed  CAS  Google Scholar 

  154. Fine JD, Weiner M, Stein A, et al. Systemic isotretinoin and recessive dystrophic epidermolysis bullosa (RDEB): results of a Phase 1 clinical trial. J Invest Dermatol. 2001;117:543.

    Google Scholar 

  155. Fine JD. Skin bioequivalents and their role in the treatment of inherited epidermolysis bullosa [Editorial]. Arch Dermatol. 2000;136:1259–60.

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Einstein Division of Dermatology, Montefiore Medical Center, Bronx, NY, USA

    Michael W. Cashman M.D.

  2. Department of Dermatology, Albert Einstein College of Medicine, Bronx, NY, USA

    Daven Doshi M.D.

  3. Dermatology, Jacobi Medical Center, Cosmetic Dermatology Clinic, Montefiore Medical Center, Albert Einstien College of Medicine, Bronx, NY, USA

    Karthik Krishnamurthy D.O.

Authors
  1. Michael W. Cashman M.D.
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  2. Daven Doshi M.D.
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  3. Karthik Krishnamurthy D.O.
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Correspondence to Karthik Krishnamurthy D.O. .

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Editors and Affiliations

  1. , Department of Dermatology, Mount Sinai School of Medicine, Front Street 220, New York, 10038, New York, USA

    Bobby Buka

  2. , Department of Dermatology, Mount Sinai School of Medicine, Front Street 220, New York, 10038, New York, USA

    Annemarie Uliasz

  3. , Dermatology, Jacobi Medical Center, Albert Einstein College of Medicine, Pelham Parkway South, Bronx, 10461, New York, USA

    Karthik Krishnamurthy

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Cashman, M.W., Doshi, D., Krishnamurthy, K. (2013). Vesiculobullous Dermatoses. In: Buka, B., Uliasz, A., Krishnamurthy, K. (eds) Buka's Emergencies in Dermatology. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-5031-3_8

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