Pemphigus Vulgaris

Key Points
Most common type of pemphigus, typically affecting interior of the mouth
Autoimmune disorder which destroys connectivity of skin cells
Consists of small easily ruptured blisters which leave behind erosions, crusting, and inflammation

Pemphigus vulgaris is the most common type of pemphigus which typically affects interior of the mouth, but may occur in other mucosa or on the skin. The condition consists of the formation of small, easily ruptured blisters. Ruptured blisters leave behind skin erosions which may crust and become red and inflamed. This may be accompanied by burning or pain in the affected area.

Pemphigus vulgaris is an autoimmune disorder in which the immune system destroys the connective tissue that holds skin cells together, causing blistering. The condition may affect any age, gender or ethnic group, but typically appears between 50-60 years of age.

Differential Diagnosis (Other conditions with similar appearance)
Dermatitis
Lupus erythematosus
Drug-Induced Bullous Disorders
Drug-induced Photosensitivity
Papular Urticaria
Epidermolysis bullosa
Pemphigus (various forms)
Epidermolysis bullosa Acquisita
Erysipelas
Erythema multiforme
Erythroderma
Fogo Selvagem
Glucagonoma Syndrome
Pseudoporphyria
Herpes simplex
Subcorneal pustular dermatosis
Impetigo
Insect Bites
Linear IgA dermatosis

Diagnosis
Key Points
Diagnosis based on skin biopsy of the affected area
Staining will be performed to confirm the condition

Pemphigus vulgaris is typically diagnosed based on a skin biopsy of the affected area, and confirmed by a stain of the affected tissue. These tests will also rule out similar appearing conditions.

Treatment
There is no effective cure for the condition
Goal of treatment is to lessen the formation of new lesions and prevent infection
Treatment typically consists of oral corticosteroids and/or immunosuppressants

There is no cure for pemphigus vulgaris. The goal of treatment is to lessen the formation of new lesions, promote healing in affected areas, and to prevent secondary infection. Oral corticosteroids are typically used in treatment, though prolonged usage and high doses carry serious risks. To reduce use of these steroids, other immunosuppressant medications are prescribed, such as azathioprine, cyclophosphamide, dapson, tetracyclines, plasmapheresis, nicotinamide, Mycophenolate mofetil, IV Immunoglobulin, gold, Infliximab, and rituximab. Proper wound care is recommended to prevent secondary infections and help encourage resolution. Affected persons will often see moderate symptoms under treatment, and it is recommended that any activity which may cause injury to the affected areas be avoided.