Background Definition

Pemphigus is an intra-epidermal autoimmune blistering disease involving the skin and mucous membranes. Pemphigus is conventionally divided into three distinct subtypes: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. Pemphigus vulgaris is characterised by oral lesions and blistering skin lesions whereas patients with pemphigus foliaceus have only skin lesions with characteristic scaling. Patients with paraneoplastic pemphigus have painful oral lesions, conjunctival reactions and a widespread erythematous, sometimes blistering, skin eruption.

In this chapter we will exclusively address pemphigus vulgaris and foliaceus, except in the discussion of studies that included paraneoplastic pemphigus patients before it was recognised as a distinct clinicopathological entity, and was thus not distinguished from other forms of pemphigus.

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