Pemphigus foliaceus

Abstract

Pemphigus foliaceus is a rare autoimmune disease that results in blistering of the skin. It is caused by autoantibodies directed against cell-surface antigens on keratinocytes, which when targeted lose their cellular adhesion properties and separate from one another to form blisters within the epidermis. The disease has two predominant types: endemic and sporadic. In pemphigus foliaceus the blisters are high in the epidermis, just below the stratum corneum, and are associated with antibodies against desmoglein-1. The disease is diagnosed based on its clinical manifestations (flaccid blisters and erosions on skin), histology (epidermal acantholysis), and immunological abnormalities (circulating and tissue-fixed antibodies against keratinocyte surface antigens). This chapter summarizes the epidemiology, clinical features and diagnostic techniques. An in-depth review of treatment modalities reported in the literature is presented and includes topical agents, anti-inflammatory agents, immunosuppressant and biologic therapy. We also present a treatment approach based on the authors' experience of treating this rare disease.