What is the prognosis of pemphigus vulgaris?

Updated: Jun 14, 2018
  • Author: Bassam Zeina, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Answer

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 dramatically. [22]

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.


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