What is the pathophysiology of pemphigus?

Pemphigus vulgaris is an autoimmune, intraepithelial, blistering disease affecting the skin and mucous membranes and is mediated by circulating autoantibodies directed against keratinocyte cell surfaces. In 1964, autoantibodies against keratinocyte surfaces were described in patients with pemphigus.

What is Cicatricial pemphigoid?

Cicatricial pemphigoid is a rare, chronic autoimmune blistering disorder which can produce scarring. It can affect the skin only, mucous membranes only, or both the skin and mucous membranes. When only mucous membranes are involved, the disease is often referred to as mucous membrane pemphigoid.

What are the different types of pemphigus?

The two main forms of pemphigus are:

• Pemphigus vulgaris is the most common type in the United States. Blisters form in the mouth and other mucosal surfaces, as well as on the skin.
• Pemphigus foliaceus is less common and only affects the skin.

Which type of hypersensitivity is pemphigus?

Pemphigus vulgaris is a type II hypersensitivity reaction, which is when the immune system produces antibodies that bind to the body’s own cells. More specifically, immune cells, called B cells produce IgG antibodies that can bind to specific desmosomes proteins – desmoglein 1 and 3.

What are the symptoms of pemphigus?

Symptoms of Pemphigus The main symptom of pemphigus is blistering of the skin and in some cases, the mucosal surfaces, such as the inside of the mouth, nose, throat, eyes, and genitals. The blisters are fragile and tend to burst, causing crusty sores.

What is Foliaceus?

Pemphigus foliaceus is an autoimmune disease that causes itchy blisters to form on your skin. It’s part of a family of rare skin conditions called pemphigus that produce blisters or sores on the skin, in the mouth, or on the genitals. There are two main types of pemphigus: pemphigus vulgaris.

What is the difference between pemphigus vulgaris and Foliaceus?

Pemphigus foliaceus produces superficial blisters confined to the skin, without involvement of mucous membranes. This is in contrast to pemphigus vulgaris where there may be extensive mucous membrane involvement (mouth, eyelids etc.). The patient with pemphigus foliaceus is usually otherwise in good health.

What is the most common form of pemphigus?

Pemphigus is a rare skin disorder characterized by blistering of your skin and mucous membranes. The most common type is pemphigus vulgaris, which involves painful sores and blisters on your skin and in your mouth.

How is pemphigus diagnosed?

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.

What is Darier’s disease?

General Discussion. Keratosis follicularis, also known as Darier disease, is a rare, genetic skin disorder. Affected individuals develop skin lesions that consist of thickened, rough bumps (papules) or plaques that may also be greasy or have a brown or yellow crust.