Pemphigus Vulgaris 

  • Author: Bassam Zeina, MD, PhD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jul 28, 2011
 

Background

Pemphigus is derived from the Greek word pemphix meaning bubble or blister. Pemphigus describes a group of chronic bullous diseases, originally named by Wichman in 1791. The term pemphigus once included most bullous eruptions of the skin, but diagnostic tests have improved, and bullous diseases have been reclassified.

The term pemphigus refers to a group of autoimmune blistering diseases of the skin and mucous membranes characterized histologically by intraepidermal blister and immunopathologically by the finding of in vivo bound and circulating immunoglobulin G (IgG) antibody directed against the cell surface of keratinocytes. The 3 primary subsets of pemphigus include pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus.[1] Each type of pemphigus has distinct clinical and immunopathologic features. Pemphigus vulgaris accounts for approximately 70% of pemphigus cases.

Next

Pathophysiology

Pemphigus vulgaris is an autoimmune, intraepithelial, blistering disease affecting the skin and mucous membranes and is mediated by circulating autoantibodies directed against keratinocyte cell surfaces. In 1964, autoantibodies against keratinocyte surfaces were described in patients with pemphigus. Clinical and experimental observations indicate that the circulating autoantibodies are pathogenic. An immunogenetic predisposition is well established.

Blisters in pemphigus vulgaris are associated with the binding of IgG autoantibodies to keratinocyte cell surface molecules. These intercellular or pemphigus vulgaris antibodies bind to keratinocyte desmosomes and to desmosome-free areas of the keratinocyte cell membrane. The binding of autoantibodies results in a loss of cell-to-cell adhesion, a process termed acantholysis. The antibody alone is capable of causing blistering without complement or inflammatory cells.

Pemphigus vulgaris antigen

Intercellular adhesion in the epidermis involves several keratinocyte cell surface molecules. Pemphigus antibody binds to keratinocyte cell surface the molecules desmoglein 1 and desmoglein 3. The binding of antibody to desmoglein may have a direct effect on desmosomal adherens or may trigger a cellular process that results in acantholysis. Antibodies specific for nondesmosomal antigens also have been described in the sera of patients with pemphigus vulgaris; however, the role of these antigens in the pathogenesis of pemphigus vulgaris is not known.

Antibodies

Patients with the mucocutaneous form of pemphigus vulgaris have pathogenic antidesmoglein 1 and antidesmoglein 3 autoantibodies. Patients with the mucosal form of pemphigus vulgaris have only antidesmoglein 3 autoantibodies. Patients with active disease have circulating and tissue-bound autoantibodies of both the immunoglobulin G1 (IgG1) and immunoglobulin G4 (IgG4) subclasses.[2, 3]

More than 80% of the patients with active disease produce autoantibodies to the desmosomal protein desmoglein. Disease activity correlates with antibody titers in most patients.[4] In patients with pemphigus vulgaris, the presence of antidesmoglein 1 autoantibodies, as determined by enzyme-linked immunosorbent assay (ELISA), is more closely correlated with the course of the disease compared with antidesmoglein 3 autoantibodies. Lack of in vivo antibody binding (reversion to a negative result on direct immunofluorescence) is the best indicator of remission and can help predict a lack of flaring when therapy is tapered.

Complement

Pemphigus antibody fixes components of complement to the surface of epidermal cells. Antibody binding may activate complement with the release of inflammatory mediators and recruitment of activated T cells. T cells are clearly required for the production of the autoantibodies, but their role in the pathogenesis of pemphigus vulgaris remains poorly understood. Interleukin 2 is the main activator of T lymphocytes, and increased soluble receptors have been detected in patients with active pemphigus vulgaris.

Previous
Next

Epidemiology

Frequency

United States

Pemphigus vulgaris is uncommon, and the exact incidence and prevalence depends on the population studied.

International

Pemphigus vulgaris has been reported to occur worldwide. Pemphigus vulgaris incidence varies from 0.5-3.2 cases per 100,000 population. Pemphigus vulgaris incidence is increased in patients of Ashkenazi Jewish descent and those of Mediterranean origin. Few familial cases have been reported.

Mortality/Morbidity

Pemphigus vulgaris is a potentially life-threatening autoimmune mucocutaneous disease with a mortality rate of approximately 5-15%.[5] Mortality in patients with pemphigus vulgaris is 3 times higher than the general population. Complications secondary to the use of high-dose corticosteroids contribute to the mortality rate. Morbidity and mortality are related to the extent of disease, the maximum dose of systemic steroids required to induce remission, and the presence of other diseases. Prognosis is worse in patients with extensive pemphigus vulgaris and in older patients.

Pemphigus vulgaris involves mucosa in 50-70% of patients. This may limit oral intake secondary to dysphagia. Blistering and erosions secondary to the rupture of blisters may be painful and may limit the patient's daily activities. Additionally, Patients with pemphigus vulgaris typically heal without scarring unless the disease is complicated by severe secondary infection.

Race

Pemphigus vulgaris affects persons of all races. The prevalence of pemphigus vulgaris is high in regions where the Jewish population is predominant.[6] For example, in Jerusalem, the prevalence of pemphigus vulgaris is estimated at 1.6 cases per 100,000 population; in Connecticut, the prevalence has been reported as 0.42 cases per 100,000 population.[7] The incidence in the United Kingdom is 0.68 case per 100 000 persons per year. The incidence of pemphigus vulgaris in Tunisia is estimated at 2.5 cases per million population per year (3.9 in women, 1.2 in men), while in France, the incidence is 1.3 cases per million population per year (no significant difference between men and women).[8] In Finland, where few people of Jewish or Mediterranean origin live, the prevalence is low, at 0.76 case per million population.[9]

Sex

The male-to-female ratio is approximately equal. In adolescence, girls are more likely to be affected than boys.

Age

The mean age of onset is approximately 50-60 years; however, the range is broad, and disease onset in older individuals and in children has been described. Patients are younger at presentation in India than in Western countries.[10]

Previous
Proceed to Clinical Presentation
 
 
Contributor Information and Disclosures
Author

Bassam Zeina, MD, PhD  Consulting Staff, Department of Dermatology, Milton Keynes Hospital, UK

Bassam Zeina, MD, PhD is a member of the following medical societies: British Association of Dermatologists, British Medical Association, and Royal Society of Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Nicole Sakka, MBBS  Foundation Year 2, Royal Liverpool and Broadgreen University Hospital, Liverpool, UK

Disclosure: Nothing to disclose.

Sohail Mansoor, MBBS, MSc  Dermatologist and Lead Physician in Dermatologic Surgery, Department of Dermatology, Barnet Hospital, UK

Sohail Mansoor, MBBS, MSc is a member of the following medical societies: American Academy of Anti-Aging Medicine, American Academy of Dermatology, American Society for Dermatologic Surgery, Royal College of Physicians and Surgeons of Glasgow, and Royal College of Physicians of the United Kingdom

Disclosure: Nothing to disclose.

Specialty Editor Board

Abby S Van Voorhees, MD  Assistant Professor, Director of Psoriasis Services and Phototherapy Units, Department of Dermatology, University of Pennsylvania School of Medicine, Hospital of the University of Pennsylvania

Abby S Van Voorhees, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, National Psoriasis Foundation, Phi Beta Kappa, Sigma Xi, and Women's Dermatologic Society

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Merck Salary Management position; Abbott Honoraria Speaking and teaching; Amgen Honoraria Review panel membership; Centocor Honoraria Consulting; Leo Consulting; Merck None Other

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD  Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Mohsin Ali, MBBS, FRCP, MRCP, to the development and writing of this article.

References

  1. Mentink LF, de Jong MC, Kloosterhuis GJ, Zuiderveen J, Jonkman MF, Pas HH. Coexistence of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. Br J Dermatol. Apr 2007;156(4):635-41. [Medline].

  2. Bhol K, Mohimen A, Ahmed AR. Correlation of subclasses of IgG with disease activity in pemphigus vulgaris. Dermatology. 1994;189 Suppl 1:85-9. [Medline].

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

  4. Fitzpatrick RE, Newcomer VD. The correlation of disease activity and antibody titers in pemphigus. Arch Dermatol. Mar 1980;116(3):285-90. [Medline].

  5. Ahmed AR, Moy R. Death in pemphigus. J Am Acad Dermatol. Aug 1982;7(2):221-8. [Medline].

  6. Pisanti S, Sharav Y, Kaufman E, Posner LN. Pemphigus vulgaris: incidence in Jews of different ethnic groups, according to age, sex, and initial lesion. Oral Surg Oral Med Oral Pathol. Sep 1974;38(3):382-7. [Medline].

  7. Simon DG, Krutchkoff D, Kaslow RA, Zarbo R. Pemphigus in Hartford County, Connecticut, from 1972 to 1977. Arch Dermatol. Sep 1980;116(9):1035-7. [Medline].

  8. 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. Feb 1995;104(2):302-5. [Medline].

  9. Hietanen J, Salo OP. Pemphigus: an epidemiological study of patients treated in Finnish hospitals between 1969 and 1978. Acta Derm Venereol. 1982;62(6):491-6. [Medline].

  10. Wilson C, Wojnarowska F, Mehra NK, Pasricha JS. Pemphigus in Oxford, UK, and New Delhi, India: a comparative study of disease characteristics and HLA antigens. Dermatology. 1994;189 Suppl 1:108-10. [Medline].

  11. Ayoub N. [Pemphigus and pemphigus-triggering drugs]. Ann Dermatol Venereol. Jun-Jul 2005;132(6-7 Pt 1):595. [Medline].

  12. Goldberg I, Ingher A, Brenner S. Pemphigus vulgaris triggered by rifampin and emotional stress. Skinmed. Sep-Oct 2004;3(5):294. [Medline].

  13. Ettlin DA. Pemphigus. Dent Clin North Am. Jan 2005;49(1):107-25, viii-ix. [Medline].

  14. Hodak E, Kremer I, David M, et al. Conjunctival involvement in pemphigus vulgaris: a clinical, histopathological and immunofluorescence study. Br J Dermatol. Nov 1990;123(5):615-20. [Medline].

  15. Trattner A, Lurie R, Leiser A, et al. Esophageal involvement in pemphigus vulgaris: a clinical, histologic, and immunopathologic study. J Am Acad Dermatol. Feb 1991;24(2 Pt 1):223-6. [Medline].

  16. Marren P, Wojnarowska F, Venning V, Wilson C, Nayar M. Vulvar involvement in autoimmune bullous diseases. J Reprod Med. Feb 1993;38(2):101-7. [Medline].

  17. Berker DD, Dalziel K, Dawber RP, Wojnarowska F. Pemphigus associated with nail dystrophy. Br J Dermatol. Oct 1993;129(4):461-4. [Medline].

  18. Engineer L, Norton LA, Ahmed AR. Nail involvement in pemphigus vulgaris. J Am Acad Dermatol. Sep 2000;43(3):529-35. [Medline].

  19. Hern S, Vaughan Jones SA, et al. Pemphigus vulgaris in pregnancy with favourable foetal prognosis. Clin Exp Dermatol. Nov 1998;23(6):260-3. [Medline].

  20. Firooz A, Mazhar A, Ahmed AR. Prevalence of autoimmune diseases in the family members of patients with pemphigus vulgaris. J Am Acad Dermatol. Sep 1994;31(3 Pt 1):434-7. [Medline].

  21. 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. Jun 1 1991;88(11):5056-60. [Medline].

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

  23. Reohr PB, Mangklabruks A, Janiga AM, DeGroot LJ, Benjasuratwong Y, Soltani K. Pemphigus vulgaris in siblings: HLA-DR4 and HLA-DQw3 and susceptibility to pemphigus. J Am Acad Dermatol. Aug 1992;27(2 Pt 1):189-93. [Medline].

  24. 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. Sep 1987;84(18):6542-5. [Medline].

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

  26. Sinha AA, Brautbar C, Szafer F, et al. A newly characterized HLA DQ beta allele associated with pemphigus vulgaris. Science. Feb 26 1988;239(4843):1026-9. [Medline].

  27. Cruz PD Jr, Coldiron BM, Sontheimer RD. Concurrent features of cutaneous lupus erythematosus and pemphigus erythematosus following myasthenia gravis and thymoma. J Am Acad Dermatol. Feb 1987;16(2 Pt 2):472-80. [Medline].

  28. Leshem YA, Katzenelson V, Yosipovitch G, David M, Mimouni D. Autoimmune diseases in patients with pemphigus and their first-degree relatives. Int J Dermatol. Jul 2011;50(7):827-31. [Medline].

  29. Helander SD, Rogers RS 3rd. The sensitivity and specificity of direct immunofluorescence testing in disorders of mucous membranes. J Am Acad Dermatol. Jan 1994;30(1):65-75. [Medline].

  30. Judd KP, Lever WF. Correlation of antibodies in skin and serum with disease severity in pemphigus. Arch Dermatol. Apr 1979;115(4):428-32. [Medline].

  31. Chams-Davatchi C, Daneshpazhooh M. Prednisolone dosage in pemphigus vulgaris. J Am Acad Dermatol. Sep 2005;53(3):547. [Medline].

  32. Tabrizi MN, Chams-Davatchi C, Esmaeeli N, et al. Accelerating effects of epidermal growth factor on skin lesions of pemphigus vulgaris: a double-blind, randomized, controlled trial. J Eur Acad Dermatol Venereol. Jan 2007;21(1):79-84. [Medline].

  33. 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. Sep 2006;55(3):449-59. [Medline].

  34. Fatourechi MM, el-Azhary RA, Gibson LE. Rituximab: applications in dermatology. Int J Dermatol. Oct 2006;45(10):1143-55; quiz 1155. [Medline].

  35. Schmidt E, Hunzelmann N, Zillikens D, Brocker EB, Goebeler M. Rituximab in refractory autoimmune bullous diseases. Clin Exp Dermatol. Jul 2006;31(4):503-8. [Medline].

  36. Schmidt E, Seitz CS, Benoit S, Brocker EB, Goebeler M. Rituximab in autoimmune bullous diseases: mixed responses and adverse effects. Br J Dermatol. Feb 2007;156(2):352-6. [Medline].

  37. el-Darouti M, Marzouk S, Abdel Hay R, et al. The use of sulfasalazine and pentoxifylline (low-cost antitumour necrosis factor drugs) as adjuvant therapy for the treatment of pemphigus vulgaris: a comparative study. Br J Dermatol. Aug 2009;161(2):313-9. [Medline].

  38. Werth VP, Fivenson D, Pandya AG, et al. Multicenter randomized, double-blind, placebo-controlled, clinical trial of dapsone as a glucocorticoid-sparing agent in maintenance-phase pemphigus vulgaris. Arch Dermatol. Jan 2008;144(1):25-32. [Medline].

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

  40. Bystryn JC, Jiao D. IVIg selectively and rapidly decreases circulating pathogenic autoantibodies in pemphigus vulgaris. Autoimmunity. Nov 2006;39(7):601-7. [Medline].

  41. Green MG, Bystryn JC. Effect of intravenous immunoglobulin therapy on serum levels of IgG1 and IgG4 antidesmoglein 1 and antidesmoglein 3 antibodies in pemphigus vulgaris. Arch Dermatol. Dec 2008;144(12):1621-4. [Medline].

  42. Mittmann N, Chan B, Knowles S, Mydlarski PR, Cosentino L, Shear N. Effect of intravenous immunoglobulin on prednisone dose in patients with pemphigus vulgaris. J Cutan Med Surg. Sep-Oct 2006;10(5):222-7. [Medline].

  43. Mydlarski PR, Ho V, Shear NH. Canadian consensus statement on the use of intravenous immunoglobulin therapy in dermatology. J Cutan Med Surg. Sep-Oct 2006;10(5):205-21. [Medline].

  44. [Best Evidence] Amagai M, Ikeda S, Shimizu H, et al. A randomized double-blind trial of intravenous immunoglobulin for pemphigus. J Am Acad Dermatol. Apr 2009;60(4):595-603. [Medline].

  45. Asarch A, Razzaque Ahmed A. Treatment of juvenile pemphigus vulgaris with intravenous immunoglobulin therapy. Pediatr Dermatol. Mar-Apr 2009;26(2):197-202. [Medline].

  46. Bakos L, Zoratto G, Brunetto L, Mazzotti N, Cartell A. Photodynamic therapy: a useful adjunct therapy for recalcitrant ulceration in pemphigus vulgaris. J Eur Acad Dermatol Venereol. May 2009;23(5):599-600. [Medline].

  47. Aberer W, Wolff-Schreiner EC, Stingl G, Wolff K. Azathioprine in the treatment of pemphigus vulgaris. A long-term follow-up. J Am Acad Dermatol. Mar 1987;16(3 Pt 1):527-33. [Medline].

  48. Baskan EB, Yilmaz M, Tunali S, Saricaoglu H. Efficacy and safety of long-term mycophenolate sodium therapy in pemphigus vulgaris. J Eur Acad Dermatol Venereol. Mar 17 2009;[Medline].

  49. Beissert S, Mimouni D, Kanwar AJ, Solomons N, Kalia V, Anhalt GJ. Treating pemphigus vulgaris with prednisone and mycophenolate mofetil: a multicenter, randomized, placebo-controlled trial. J Invest Dermatol. Aug 2010;130(8):2041-8. [Medline].

  50. Strowd LC, Taylor SL, Jorizzo JL, Namazi MR. Therapeutic ladder for pemphigus vulgaris: emphasis on achieving complete remission. J Am Acad Dermatol. Mar 2011;64(3):490-4. [Medline].

  51. Ahmed AR, Spigelman Z, Cavacini LA, Posner MR. Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin. N Engl J Med. Oct 26 2006;355(17):1772-9. [Medline].

  52. Lolis M, Toosi S, Czernik A, Bystryn JC. Effect of intravenous immunoglobulin with or without cytotoxic drugs on pemphigus intercellular antibodies. J Am Acad Dermatol. Mar 2011;64(3):484-9. [Medline].

  53. Jackson AP, Hall AG, McLelland J. Thiopurine methyltransferase levels should be measured before commencing patients on azathioprine. Br J Dermatol. Jan 1997;136(1):133-4. [Medline].

  54. Snow JL, Gibson LE. The role of genetic variation in thiopurine methyltransferase activity and the efficacy and/or side effects of azathioprine therapy in dermatologic patients. Arch Dermatol. Feb 1995;131(2):193-7. [Medline].

  55. Tavadia SM, Mydlarski PR, Reis MD, et al. Screening for azathioprine toxicity: a pharmacoeconomic analysis based on a target case. J Am Acad Dermatol. Apr 2000;42(4):628-32. [Medline].

  56. [Guideline] Anstey AV, Wakelin S, Reynolds NJ. Guidelines for prescribing azathioprine in dermatology. Br J Dermatol. Dec 2004;151(6):1123-32. [Medline].

  57. Amagai M, Klaus-Kovtun V, Stanley JR. Autoantibodies against a novel epithelial cadherin in pemphigus vulgaris, a disease of cell adhesion. Cell. Nov 29 1991;67(5):869-77. [Medline].

  58. Anhalt GJ, Diaz LA. Pemphigus vulgaris--a model for cutaneous autoimmunity. J Am Acad Dermatol. Jul 2004;51(1 Suppl):S20-1. [Medline].

  59. Bystryn JC, Moore MM. Cutaneous pemphigus vulgaris: what causes it?. J Am Acad Dermatol. Jul 2006;55(1):175-6; author reply 176-7. [Medline].

  60. Bystryn JC, Steinman NM. The adjuvant therapy of pemphigus. An update. Arch Dermatol. Feb 1996;132(2):203-12. [Medline].

  61. Carson PJ, Hameed A, Ahmed AR. Influence of treatment on the clinical course of pemphigus vulgaris. J Am Acad Dermatol. Apr 1996;34(4):645-52. [Medline].

  62. Dmochowski M, Hashimoto T, Amagai M, et al. The extracellular aminoterminal domain of bovine desmoglein 1 (Dsg1) is recognized only by certain pemphigus foliaceus sera, whereas its intracellular domain is recognized by both pemphigus vulgaris and pemphigus foliaceus sera. J Invest Dermatol. Aug 1994;103(2):173-7. [Medline].

  63. Dmochowski M, Hashimoto T, Chidgey MA, et al. Demonstration of antibodies to bovine desmocollin isoforms in certain pemphigus sera. Br J Dermatol. Oct 1995;133(4):519-25. [Medline].

  64. Elder D, Elenitsas R, Jaworsky C, Johnson BL. Pemphigus vulgaris. In: Lever's Histopathology of the Skin. 8th ed. Lippincott Williams & Wilkins; 1997:218-50.

  65. Grando SA, Glukhenky BT, Drannik GN, Epshtein EV, Kostromin AP, Korostash TA. Mediators of inflammation in blister fluids from patients with pemphigus vulgaris and bullous pemphigoid. Arch Dermatol. Jul 1989;125(7):925-30. [Medline].

  66. Grando SA, Terman AK, Stupina AS, Glukhenky BT, Romanenko AB. Ultrastructural study of clinically uninvolved skin of patients with pemphigus vulgaris. Clin Exp Dermatol. Sep 1991;16(5):359-63. [Medline].

  67. [Guideline] Harman KE, Albert S, Black MM. Guidelines for the management of pemphigus vulgaris. Br J Dermatol. Nov 2003;149(5):926-37. [Medline].

  68. Hashimoto T. Recent advances in the study of the pathophysiology of pemphigus. Arch Dermatol Res. Apr 2003;295 Suppl 1:S2-11. [Medline].

  69. Hashimoto T, Ogawa MM, Konohana A, Nishikawa T. Detection of pemphigus vulgaris and pemphigus foliaceus antigens by immunoblot analysis using different antigen sources. J Invest Dermatol. Mar 1990;94(3):327-31. [Medline].

  70. Jin P, Shao C, Ye G. Chronic bullous dermatoses in China. Int J Dermatol. Feb 1993;32(2):89-92. [Medline].

  71. Kawana S, Geoghegan WD, Jordon RE, Nishiyama S. Deposition of the membrane attack complex of complement in pemphigus vulgaris and pemphigus foliaceus skin. J Invest Dermatol. Apr 1989;92(4):588-92. [Medline].

  72. Kirtschig G, Wojnarowska F. Autoimmune blistering diseases: an up-date of diagnostic methods and investigations. Clin Exp Dermatol. Mar 1994;19(2):97-112. [Medline].

  73. Korman NJ. Pemphigus. Dermatol Clin. Oct 1990;8(4):689-700. [Medline].

  74. Krain LS. Pemphigus. Epidemiologic and survival characteristics of 59 patients, 1955-1973. Arch Dermatol. Dec 1974;110(6):862-5. [Medline].

  75. Loo WJ, Burrows NP. Management of autoimmune skin disorders in the elderly. Drugs Aging. 2004;21(12):767-77. [Medline].

  76. Mimouni D, Nousari CH, Cummins DL, Kouba DJ, David M, Anhalt GJ. Differences and similarities among expert opinions on the diagnosis and treatment of pemphigus vulgaris. J Am Acad Dermatol. Dec 2003;49(6):1059-62. [Medline].

  77. Rosenberg FR, Sanders S, Nelson CT. Pemphigus: a 20-year review of 107 patients treated with corticosteroids. Arch Dermatol. Jul 1976;112(7):962-70. [Medline].

  78. Savin JA. Some factors affecting prognosis in pemphigus vulgaris and pemphigoid. Br J Dermatol. Apr 1981;104(4):415-20. [Medline].

  79. 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. [Medline].

 
Previous
Next
 
Early, small blister filled with clear fluid arises on healthy skin.
Flaccid blister filled with clear fluid arises on healthy skin.
An erosion.
Erosions and healing areas on the back.
Healing areas on the chest and abdomen.
Direct immunofluorescence showing intercellular immunoglobulin G throughout the epidermis of a patient with pemphigus vulgaris.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.