Pemphigus vulgaris

Pemphigus vulgaris is an uncommon, potentially fatal, autoimmune disorder characterized by intraepidermal blisters and extensive erosions on apparently healthy skin and mucous membranes. Diagnosis is by skin biopsy with direct and indirect immunofluorescence and enzyme-linked immunosorbent assay (ELISA) testing. Treatment is with corticosteroids and sometimes other immunosuppressive therapies.

 Pemphigus vulgaris 

Flaccid bullae, which are the primary lesions of pemphigus vulgaris, cause widespread and painful skin, oral, and other mucosal erosions. About half of patients have only oral erosions, which rupture and remain as chronic, painful lesions for variable periods. Often, oral lesions precede skin involvement. Dysphagia and poor oral intake are common because lesions also may occur in the upper esophagus. Cutaneous bullae typically arise in normal-appearing skin, rupture, and leave a raw area with crusting. Itching is usually absent. Erosions often become infected. If large portions of the body are affected, fluid and electrolyte loss may be significant.

More details: 📖 Dermatopathology A-Z: A Comprehensive Guide 

To establish a diagnosis of pemphigus vulgaris, perform the following tests:

- Histopathology from the edge of a blister

- Direct immunofluorescence (DIF) on normal-appearing perilesional skin.

Indirect immunofluorescence (IDIF) using the patient's serum if DIF results are positive: The preferred substrate for IDIF is monkey esophagus or salt-split normal human skin substrate.

Direct immunofluorescence showing intercellular immunoglobulin G throughout
the epidermis of a patient with pemphigus vulgaris

DIF demonstrates in vivo deposits of antibodies and other immunoreactants, such as complement. DIF usually shows immunoglobulin G (IgG) deposited on the surface of the keratinocytes in and around lesions. IgG1 and IgG4 are the most common subclasses. Complement components such as C3 and immunoglobulin M are present less frequently than IgG. DIF shows intercellular deposition throughout the epidermis. This pattern of immunoreactants is not specific for pemphigus vulgaris and may be seen in pemphigus vegetans, pemphigus foliaceus, and pemphigus erythematosus. The best location for DIF testing is on normal perilesional skin. When DIF testing is performed on lesional skin, false-positive results can be observed. 

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