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The term pemphigus refers to a group of autoimmune blistering diseases of skin and mucous membranes that are characterized histologically by intraepidermal blisters due to acantholysis (i.e., separation of epidermal cells from each other) and immunopathologically by in vivo bound and circulating immunoglobulin (Ig) directed against the cell surface of keratinocytes. The nosology of this group of diseases is outlined in Box 54-1. Essentially, pemphigus can be divided into four major types: (1) vulgaris, (2) foliaceus, (3) paraneoplastic (see Chapter 55), and (4) IgA pemphigus (see Chapter 54). In pemphigus vulgaris (PV), the blister occurs in the deeper part of the epidermis, just above the basal layer, and in pemphigus foliaceus (PF), also called superficial pemphigus, the blister is in the granular layer.
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The history of the discovery of pemphigus, and its various forms, is covered in Walter Lever's classic monograph Pemphigus and Pemphigoid.1 Both PV and PF display a spectrum of disease. Various points along these spectra have been given unique names, but because the presentation of these diseases is fluid, patients’ disease usually crosses these artificial designations over time. Thus, patients with PV may present with more localized disease, one form of which is called pemphigus vegetans of Hallopeau. This may become slightly more extensive and may merge into pemphigus vegetans of Neumann. Finally, with more severe disease, full-blown PV may appear. Similarly, ...