**12. Conclusion**

PV and PF are debilitating and potentially life-threatening conditions that are therefore important to promptly recognize clinically and then confirm through their characteristic features on histology and direct immunofluorescence. The detection of serum autoantibodies by indirect immunofluorescence or ELISA does not obviate the need for a tissue diagnosis but might be useful is assessing disease severity and activity.

Although, corticosteroids remain the cornerstone of therapy for pemphigus, the morbidity associated with its use restricts its value as a long term treatment option. This is complicated by the fact that steroid-sparing agents are also associated with serious adverse events and there are only few randomized controlled trials demonstrating a beneficial response from the use of these agents. This has been further compounded, until very recently, by the inconsistent parameters of disease activity used in different studies. Azathioprine and mycophenolate mofetil appear to be the most feasible first line adjunctive agents in terms of inducing and maintaining remission and having a comparatively favourable side effect profile. An enhanced understanding of the pathogenesis of pemphigus has resulted in the implementation of a number of novel agents. These therapies have also been mainly studies through case series reports, are expensive and difficult to access in some centres, and are associated with a number of deleterious side effects. Rituximab, has emerged as the therapy of choice in severe refractory disease and is now being explored as a first line agent. We eagerly await further studies on the effects and safety profiles of more specific agents, especially those targeting signalling molecules involved in the pathogenesis of pemphigus.

We have formulated guidelines on the treatment of pemphigus as suggested by the current level of evidence [204]. Corticosteroids remain the mainstay of treatment and should be initiated at a dose of 0.5 mg /kg of oral prednisone per day and continued at this dose until disease control is obtained. This is defined as the time at which new lesions cease to form and existing lesions begin to heal and in responsive patients, this usually occurs within weeks. At the end of the consolidation phase defined as the point in time where no new lesions have occurred after 2 weeks and 80% of established lesions have healed, the corticosteroid dose is tapered. Unless disease is mild we would recommend adding an adjuvant agent. Either mycophenolate mofetil or azathioprine could be utilized at this stage. For severe or recalcitrant disease rituximab is recommended given the current level of evidence. Other biologic agents, extracorporeal therapies or cytotoxic agents can be considered if rituximab is unavailable or contraindicated.
