Prognosis

Pemphigus vulgaris (PV) and pemphigus foliaceus (PF)

The outlook for patients with PV and PF is good if the disease is adequately controlled. There are cases of spontaneous remission, but most patients require some form of long-term immunosuppression.[39]

Rituximab induces complete remission off therapy in nearly 90% of patients.[34]​

The importance of early and aggressive treatment is supported by the fact that patients with incomplete treatment (i.e., those who have multiple remissions on subtherapeutic therapy) often develop more aggressive disease. In this process, referred to as disease-hardening, more indolent disease becomes resistant to treatment.

Paraneoplastic pemphigus (PNP)

PNP is more difficult to treat, and mortality can approach 90%.[13][32]​​ The best prognosis is in isolated cases where the underlying malignancy remits through surgical or medical therapy, but many patients continue to have progression of their PNP despite treatment of their underlying malignancy, PNP, or both. Some patients may have aggressive progression of their PNP despite clinical remission of their underlying cancer.

Treatment may be inappropriately delayed in patients with PNP upon achieving cancer remission, because there is a perception (incorrect) that eradication of the underlying malignancy is curative. There is also a valid concern that aggressive immunosuppression could lead to recurrence of the original cancer.

Patients with PNP with erythema multiforme-like skin lesions and keratinocyte necrosis were found to have a poorer prognosis than those who had PNP without these skin findings.[31] The most common causes of death in patients with PNP include severe infections due to immunosuppressive treatment, bronchiolitis obliterans-related respiratory failure, and progression of the underlying malignancy.[13]​[31]​[32]​

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