eMedicine Specialties > Dermatology > Bullous Diseases

Pemphigus Vulgaris: Follow-up

Author: Bassam Zeina, MD, PhD, Consulting Staff, Department of Dermatology, Milton Keynes Hospital, UK
Coauthor(s): Sohail Mansoor, MBBS, MSc, Dermatologist and Lead Physician in Dermatologic Surgery, Department of Dermatology, Barnet Hospital, UK
Contributor Information and Disclosures

Updated: Aug 14, 2009

Follow-up

Complications

  • Secondary infection, which may be either systemic or localized to the skin, may occur because of the use of immunosuppressants and the presence of multiple erosions. Cutaneous infection delays wound healing and increases the risk of scarring.
  • Malignancies resulting from immunosuppressants have been reported.
  • Growth retardation has been reported in children taking systemic corticosteroids and immunosuppressants.
  • Bone marrow suppression has been reported in patients receiving immunosuppressants. Increased incidence is reported of leukemia and lymphoma in patients receiving prolonged immunosuppression.
  • Impaired immune responsiveness caused by corticosteroids and other immunosuppressive drugs may result in the rapid spread of infection. Corticosteroids suppress clinical signs of infection and may allow diseases such as septicemia or tuberculosis to reach an advanced stage before diagnosis.
  • Osteoporosis may occur following the use of systemic corticosteroids.
  • Adrenal insufficiency has been reported following prolonged use of glucocorticoids.

Prognosis

  • The severity and natural history of pemphigus vulgaris (PV) are variable, but before the advent of steroids, most patients with PV died. Treatment with systemic steroids has reduced the mortality rate to 5-15%.
  • Most deaths occur during the first few years of disease, and if the patient survives 5 years, the prognosis is good. Early disease probably is easier to control than widespread disease, and mortality may be higher if therapy is delayed.
  • Morbidity and mortality are related to the extent of disease, the maximum dose of prednisolone required to induce remission, and the presence of other diseases. The outlook is worse in older patients and in patients with extensive disease.

Patient Education

  • Minimize trauma to the skin because the patient's skin is fragile both from the disease and from the use of topical and systemic steroids.
  • The patient's understanding of the disease and education is important because of the chronic nature of this disorder.
  • Educate patients regarding their medications. They should know about dose, adverse effects, and symptoms of toxicity so they can report adverse effects to the physician.
  • Educate patients about appropriate wound care.

Miscellaneous

Medicolegal Pitfalls

  • Failure to secure the correct diagnosis using a compatible history and physical and appropriate testing. Routine histology and immunofluorescence should be performed to confirm the diagnosis and prior to treatment.
  • Failure to explain adverse effects of treatment to patients on systemic therapy. Patients must be monitored appropriately for adverse effects, toxicity, and response to treatment.

Special Concerns

  • An accurate diagnosis is important before starting the treatment. Pemphigus vulgaris (PV) shares some common features with other autoimmune blistering diseases, which sometimes makes the diagnosis difficult. Criteria for diagnosis include the following:
    • Compatible clinical picture
    • Intradermal blistering disease confirmed by histopathology
    • Intercellular immunofluorescence throughout the epidermis of immunoglobulin and complement components confirmed by DIF and IDIF
  • Physicians should educate patients about the disease, adverse effects, risks, drug interactions, and contraindications of the medications.
  • Therapeutic regimen is chosen for each individual patient after assessing the risk and benefits for each medication.
 
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.



More on Pemphigus Vulgaris

Overview: Pemphigus Vulgaris
Differential Diagnoses & Workup: Pemphigus Vulgaris
Treatment & Medication: Pemphigus Vulgaris
Follow-up: Pemphigus Vulgaris
Multimedia: Pemphigus Vulgaris
References
Further Reading
[ CLOSE WINDOW ]

References

  1. 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].

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

  3. 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].

  4. 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].

  5. 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].

  6. 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].

  7. 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].

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

  9. 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].

  10. 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].

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

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

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

  14. 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].

  15. 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].

  16. 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].

  17. Reohr PB, Mangklabruks A, Janiga AM, et al. 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].

  18. 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].

  19. 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].

  20. 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].

  21. Tabrizi M, 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].

  22. [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].

  23. 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].

  24. 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].

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

  26. 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].

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

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

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

  30. 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].

  31. 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].

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

  33. 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].

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

  35. 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].

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

  37. 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].

  38. 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].

  39. 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].

  40. el-Darouti M, Marzouk S, Abdel Hay R, el-Tawdy A, Fawzy M, Leheta T, 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].

  41. 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.

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

  43. Fatourechi MM, El-Azhary RA, Gibson LE. Rituximab: Applications in dermatology. Int J Dermatol. Oct 2006;45(10):1143-55. [Medline].

  44. Grando SA, Glukhenky BT, Drannik GN, et al. Mediators of inflammation in blister fluids from patients with pemphigus vulgaris and bullous pemphigoid. Arch Dermatol. Jul 1989;125(7):925-30. [Medline].

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

  46. 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].

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

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

  49. 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].

  50. 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].

  51. 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].

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

  53. 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].

  54. 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].

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

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

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

  58. Marren P, Wojnarowska F, Venning V, et al. Vulvar involvement in autoimmune bullous diseases. J Reprod Med. Feb 1993;38(2):101-7. [Medline].

  59. 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].

  60. Mentink LF, de Jong MC, Kloosterhuis GJ, et al. Coexistence of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. Br J Dermatol. Jan 29 2007;[Medline].

  61. Mimouni D, Nousari CH, Cummins DL, et al. 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].

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

  63. 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].

  64. 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].

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

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

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

  68. 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].

  69. 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].

  70. 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].

  71. 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].

  72. Werth VP, Fivenson D, Pandya AG, Chen D, Rico MJ, Albrecht J, 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].

  73. 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].

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

[ CLOSE WINDOW ]

Keywords

pemphigus vulgaris, PV, bullous disease

[ CLOSE WINDOW ]

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)

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.

Medical Editor

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; Genentech Honoraria Consulting; Incyte Grant/research funds Other; Warner Chilcott Honoraria Consulting; Merck Salary Management position; Abbott  Speaking and teaching

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University 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.

Managing Editor

Jeffrey P Callen, MD, Professor of Medicine, 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 Honoraria Consulting; Centocor Honoraria Consulting; Medicis Honoraria Consulting; Celgene Honoraria Consulting

CME Editor

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 None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
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.