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): Nicole Sakka, MBBS, Foundation Year 2, Royal Liverpool and Broadgreen University Hospital, Liverpool, UK; Sohail Mansoor, MBBS, MSc, Dermatologist and Lead Physician in Dermatologic Surgery, Department of Dermatology, Barnet Hospital, UK
Contributor Information and Disclosures

Updated: Feb 3, 2010

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.
  • Long-term immunosuppressant therapy may result in infections and secondary malignancies (eg, Kaposi sarcoma), owing to impaired immune surveillance.
  • Growth retardation has been reported in children taking systemic corticosteroids and immunosuppressants.
  • Bone marrow suppression has been reported in patients receiving immunosuppressants. An increased incidence of leukemia and lymphoma is reported 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 are variable, but before the advent of steroids, most patients with pemphigus vulgaris died. Treatment with systemic steroids has reduced the mortality rate to 5-15%.
  • Untreated, pemphigus vulgaris is often fatal because of the susceptibility to infection and fluid and electrolyte disturbances.
  • 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 rates 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.
  • Prognosis is usually better in childhood than in adulthood.
  • A few rare cases of pemphigus vulgaris transitioning to pemphigus foliaceus have been reported.

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 about pemphigus vulgaris 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 testing should be performed to confirm the diagnosis 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 treatment. Pemphigus vulgaris 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 direct immunofluorescence and indirect immunofluorescence
  • Physicians should educate patients about the disease, adverse effects, risks, drug interactions, and contraindications of the medications.
  • The 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
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Keywords

pemphigus vulgaris, pemphigus, pemphigoid, pemphigus treatment, bullous disease, chronic bullous disease

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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.

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 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, 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.

 
 
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