**Author details**

transfer murine model of pemphigus vulgaris [195]. It has been demonstrated that p53 knockout mice are protected from PV IgG induced disease [196]. Neonatal mice pretreated with p53 inhibitor pifithrin-alpha were resistant to both PV and PF IgG mediated

As shown by the studies of Waschke et al, PV autoantibodies directly block desmoglein-3 transinteraction in contrast to anti-desmoglein antibodies found in PF, which disrupt desmoglein-1 transinteraction via cellular signalling events rather than by direct inhibi‐ tion [198]-[200]. Hence targeting these signalling proteins in PF may provide a more specific target of therapy as compared to immunosuppressive or biological agents. It remains to be determined whether targeting these signalling proteins results in mediating disease remission in humans. Although they target specific areas of pemphigus pathogenesis, these proteins mediate numerous cellular functions and hence the outcome of early safety studies

The elimination or avoidance of an antigen in a genetically susceptible individual may prevent the onset of disease or reduce disease activity. Cessation of offending medica‐ tions such as penicillamine or captopril results in remission of drug-induced PF [25],[201], [202]. Similarly, patients with endemic pemphigus, who relocate from their native rural endemic environment to a more industrialized area experience clinical and immunologic disease regression [203]. Hence, the clear identification of a triggering environmental antigen such as arthropod protein, microorganism or otherwise, will not only enhance our understanding of disease pathogenesis but also significantly enhance therapies in endem‐

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

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

blister formation [197].

132 Skin Biopsy - Diagnosis and Treatment

is eagerly awaited.

**12. Conclusion**

**11. Elimination of triggering antigens**

ic pemphigus and possibly non-endemic PF [5].

might be useful is assessing disease severity and activity.

Suran L. Fernando1,2, Jamma Li1 and Mark Schifter3,4

1 Department of Clinical Immunology and Allergy, Royal North Shore Hospital, Sydney, Australia

2 Sydney Medical School – Northern, Sydney University, Sydney, Australia

3 Department of Oral Medicine, Oral Pathology and Special Care Dentistry, Westmead Hos‐ pital, (Western) Sydney, Australia

4 Faculty of Dentistry, University of Sydney, Sydney, Australia
