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AT-A-GLANCE

AT-A-GLANCE

  • Two major types: pemphigus vulgaris and pemphigus foliaceus.

  • Pemphigus vulgaris: erosions on mucous membranes and skin; flaccid blisters on skin.

  • Pemphigus foliaceus: crusted, scaly skin lesions.

  • Pemphigus vulgaris histology: suprabasal acantholysis.

  • Pemphigus foliaceus histology: subcorneal acantholysis.

  • Autoantigens are desmogleins, transmembrane desmosomal adhesion molecules.

  • Diagnosis depends on histology showing intraepidermal acantholysis, immunofluorescence studies documenting the presence of cell surface autoantibodies, either bound to patient skin or in the serum, and/or enzyme-linked immunosorbent assay showing anti-desmoglein antibodies

  • Therapy includes topical and systemic corticosteroids, oral immunosuppressive agents, intravenous immunoglobulin, and rituximab (anti-CD20 monoclonal antibody).

INTRODUCTION

DEFINITIONS

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 (ie, separation of epidermal cells from each other) and immunopathologically by in vivo bound and circulating immunoglobulin directed against the cell surface of keratinocytes. The nosology of this group of diseases is outlined in Table 52-1. Essentially, pemphigus can be divided into 4 major types: vulgaris, foliaceus (Table 52-2), paraneoplastic (Chap. 53), and IgA pemphigus (Chap. 57). 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.

Table 52-1Nosology and Differential Diagnosis of Pemphigus
Table 52-2Pemphigus Subtypes and Variants

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