Pemphigus

Incidence in USA: 0.1–0.5:100,000 per y for pemphigus vulgaris (the most common form of pemphigus)

Individuals from ages 40-60 y most commonly affected.

Infection, sepsis

Electrolyte abnormalities and dehydration, with extensive lesions

Volume status with oropharyngeal lesions and decreased oral intake

Skin and/or pharyngeal blisters (lesions may be limited to the oropharynx), sloughing of mucosa, bleeding produced by airway manipulation

Consequences of steroid treatment (e.g., hypertension, hyperglycemia, gastric or duodenal ulceration, myopathy, infection, psychic disturbances, osteoporosis) or immunosuppressive therapy (bone marrow suppression, susceptibility to opportunistic infections and cancer)

An autoimmune intraepidermal blistering disease of the skin and mucous membranes. Oral lesions are most common. Blisters rupture easily but heal slowly.

There are four types: Pemphigus vulgaris (most common and severe form), pemphigus pemphigus foliaceus, IgA pemphigus, and paraneoplastic pemphigus.

The 5-y mortality is 5–15% for treated pemphigus vulgaris. The most common cause of death is infection, usually with Staphylococcus aureus.

Occasionally pemphigus can coexist with other autoimmune diseases, a thymoma (with or without myasthenia gravis), or malignancies.