eMedicine Specialties > Dermatology > Bullous Diseases
Pemphigus Vulgaris: Follow-up
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.
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 |
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| Differential Diagnoses & Workup: Pemphigus Vulgaris |
| Treatment & Medication: Pemphigus Vulgaris |
Follow-up: Pemphigus Vulgaris |
| Multimedia: Pemphigus Vulgaris |
| References |
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Keywords
pemphigus vulgaris, pemphigus, pemphigoid, pemphigus treatment, bullous disease, chronic bullous disease
Follow-up: Pemphigus Vulgaris