Pemphigus herpetiformis

Abstract

Pemphigus herpetiformis is a rare variant of pemphigus first described almost 40 years ago. Clinical features overlapping with dermatitis herpetiformis but histopathologically and immunologically consistent with pemphigus are typical. Diagnosis is often delayed due to the unusual presentation and absence of blisters. Initial reports implicated antibodies to desmoglein-1 (Dsg1) as key in pathogenesis. It is increasingly clear this is not universally the case and may include Dsg-3 and/or desmocollin antibodies. Although treatment is based on small case series reports suggest a combination approach of corticosteroid and dapsone may be most appropriate with other options including azathioprine, methotrexate or mycophenolate mofetil. The response to rituximab remains to be established. This chapter provides an overview of pemphigus herpetiformis, summarises treatment options and provides a proposed strategy for management.