Pemphigus 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.
- Diagnosis depends on histology showing intraepidermal acantholysis and immunofluorescence studies documenting the presence of cell surface autoantibodies, either bound to patient skin or in the serum.
- Pemphigus vulgaris histology: suprabasal acantholysis.
- Pemphigus foliaceus histology: subcorneal acantholysis.
- Direct immunofluorescence shows immunoglobulin G (IgG) on the keratinocyte cell surface of the patient's skin; indirect immunofluorescence shows IgG in patient serum that binds the cell surface of normal keratinocytes.
- Autoantigens are desmogleins, transmembrane desmosomal adhesion molecules.
- Therapy includes topical and systemic corticosteroids and immunosuppressive agents.
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.
Box 54-1 Differential Diagnosis of Pemphigus |Favorite Table|Download (.pdf)
Box 54-1 Differential Diagnosis of Pemphigus
- Pemphigus vulgaris
- Pemphigus vegetans
- Pemphigus foliaceus
- Pemphigus erythematosus
- Endemic pemphigus foliaceus (e.g., fogo selvagem)
- Immunoglobulin A (IgA) pemphigus
- Subcorneal pustular dermatosis
- Intraepidermal neutrophilic dermatosis
- Paraneoplastic pemphigus
INTRAEPIDERMAL BLISTERING DISEASES WITHOUT AUTOANTIBODIES
- Familial benign pemphigus (Hailey–Hailey disease)
- Bullous impetigo, staphylococcal scalded-skin syndrome
- Blisters from herpes simplex and zoster
- Allergic contact dermatitis (e.g., rhus dermatitis)
- Epidermolysis bullosa simplex
- Incontinentia pigmenti
MOUTH ULCERS/EROSION WITHOUT AUTOANTIBODIES
- Aphthous ulcers
- Lichen planus
- Behçet disease
SUBEPIDERMAL BLISTERING DISEASES WITH AUTOANTIBODIES
- Bullous pemphigoid
- Herpes gestationis
- Cicatricial pemphigoid
- Epidermolysis bullosa acquisita
- Linear IgA disease and chronic bullous disease of childhood
- Dermatitis herpetiformis
- Bullous lupus erythematosus
SUBEPIDERMAL BLISTERING DISEASES WITHOUT AUTOANTIBODIES
- Erythema multiforme
- Toxic epidermal necrolysis
- Junctional or dystrophic epidermolysis bullosa
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, ...