**7. Treatment**

Therapy has resulted in a drastic decline of the mortality from pemphigus rate to below 7% [24],[47]; however, mortality still occurs, predominantly iatrogenic, caused by complications of the immunosuppressive therapy [24],[47]. PF tends to have a relatively benign course compared with PV. However, mortality rates of untreated endemic PF are still very high, ranging from 40 to 60% [48],[49]. With appropriate treatment endemic PF has a mortality rate of less than 10% [48]. The mortality of PV was 75% before the introduction of corticosteroids in the early 1950s [47]. Studies that differentiate PV according to clinical phenotype have shown a lower mortality in patients with predominantly mucosal PV (1–17%) compared with those with mucocutaneous PV (34–42%)[24]. Hence, the goal of managing patients with pemphigus is inducing and maintaining remission using those evidenced-based treatments that have a favourable side effect profile.

Pemphigus is a rare disease and therefore it is not surprising that only a few blinded random‐ ized controlled trials have been performed to guide treatment decisions. A 2009 Cochrane review [50] assessed interventions for PV and PF and concluded that there is inadequate information available to ascertain optimal therapy for pemphigus. They ascertained that the quality of most studies was not high and the majority examine patients with newly diagnosed or active disease. Another consideration in the evaluation of data is lack of generally accepted definitions and measurements for the clinical evaluation of patients with pemphigus and the definitions of disease control and remission. A recent consensus statement has been released to assist with future trials enabling improved comparisons to be made [51].

As high-dose systemic corticosteroids, followed by alternate immune-suppressive agents, serves as the mainstay of initial therapy for PV, there is the need to exclude underlying latent infectious diseases that can be reactivated by the corticosteroids (e.g.: HIV, Hepatitis B and C, and tuberculosis). In addition, screening for the diseases initiated or exacerbated by high-dose 'steroids, such as hypertension, diabetes mellitus and osteoporosis is prudent.
