PemphigusPemphigus is a group of organ specific autoimmune diseases characterized by the production of autoantibodies to desmogleins. They are mucocutaneous blistering diseases that demonstrate a loss of cohesion of the epidermal cells (acantholysis) which result in the formation of clefts in the epidermis. Autoantibodies directed against adhesion molecules cause epidermal keratinocytes to separate, resulting in intraepidermal bullae. There are two histological types of pemphigus: deep (e.g., pemphigus vulgaris) and superficial (e.g., pemphigus foliaceus). They differ in the epidermal layers that are affected, in the clinical manifestations of the diseases, and in the associated immunologic abnormalities. Pemphigus has an incidence of 1-10 new cases per 1 million people worldwide, with variations in different regions and among different ethnic groups. Incidence is increased in patients of Ashkenazi Jewish descent and those of Mediterranean origin as high as 16 to 32 cases per million people. is a group of rare
autoimmuneIn an autoimmune disease, the body's immune system attacks its own organs or tissues by producing autoantibodiesantibodies against the self (antibodies against the self). blistering diseases of the skin and/or mucous membranes.
PemphigusPemphigus is a group of organ specific autoimmune diseases characterized by the production of autoantibodies to desmogleins. They are mucocutaneous blistering diseases that demonstrate a loss of cohesion of the epidermal cells (acantholysis) which result in the formation of clefts in the epidermis. Autoantibodies directed against adhesion molecules cause epidermal keratinocytes to separate, resulting in intraepidermal bullae. There are two histological types of pemphigus: deep (e.g., pemphigus vulgaris) and superficial (e.g., pemphigus foliaceus). They differ in the epidermal layers that are affected, in the clinical manifestations of the diseases, and in the associated immunologic abnormalities. Pemphigus has an incidence of 1-10 new cases per 1 million people worldwide, with variations in different regions and among different ethnic groups. Incidence is increased in patients of Ashkenazi Jewish descent and those of Mediterranean origin as high as 16 to 32 cases per million people. is chronic and progressive and characterised by blisters and ulcers on the skin and mucous membranes.
The oral mucosa is often the first site of involvement. Typically patients will have had multiple oral ulcers (broken blisters) that persist for weeks to months. Pemphigus Vulgaris affects the oral mucosa in nearly all cases (>80%) and affects the oral mucosa first in the majority of cases (c), and may in fact be the only site in patients.
A minority will present only with cutaneous erosions. Skin involved is typically the upper chest, back, scalp, and face, but lesions can occur on any part of the body. Note that the skin dominant form manifests the Dsg1 autoantibody profile (as in pemphigusPemphigus is a group of organ specific autoimmune diseases characterized by the production of autoantibodies to desmogleins. They are mucocutaneous blistering diseases that demonstrate a loss of cohesion of the epidermal cells (acantholysis) which result in the formation of clefts in the epidermis. Autoantibodies directed against adhesion molecules cause epidermal keratinocytes to separate, resulting in intraepidermal bullae. There are two histological types of pemphigus: deep (e.g., pemphigus vulgaris) and superficial (e.g., pemphigus foliaceus). They differ in the epidermal layers that are affected, in the clinical manifestations of the diseases, and in the associated immunologic abnormalities. Pemphigus has an incidence of 1-10 new cases per 1 million people worldwide, with variations in different regions and among different ethnic groups. Incidence is increased in patients of Ashkenazi Jewish descent and those of Mediterranean origin as high as 16 to 32 cases per million people. foliaceus), whereas the mucosally dominant form manifests the Dsg3 autoantibody profile. A mixed pattern of skin and mucosa manifests a mixed Dsg1 and Dsg3 autoantibody profile. The condition progresses over weeks to months.
Sites often overlooked include around the nails (manifest as painful, red, and swollen), the pharynx and larynx (pain on swallowing and hoarseness), and the nasal cavity (nasal congestion and a bloody mucous discharge, particularly noticeable upon blowing the nose in the morning). As many as 49% of patients were shown to have laryngeal and nasal involvement (a).
Antibodies that attack one’s own cells are called
autoantibodiesantibodies against the self. The part of the cells that are attacked in
pemphigusPemphigus is a group of organ specific autoimmune diseases characterized by the production of autoantibodies to desmogleins. They are mucocutaneous blistering diseases that demonstrate a loss of cohesion of the epidermal cells (acantholysis) which result in the formation of clefts in the epidermis. Autoantibodies directed against adhesion molecules cause epidermal keratinocytes to separate, resulting in intraepidermal bullae. There are two histological types of pemphigus: deep (e.g., pemphigus vulgaris) and superficial (e.g., pemphigus foliaceus). They differ in the epidermal layers that are affected, in the clinical manifestations of the diseases, and in the associated immunologic abnormalities. Pemphigus has an incidence of 1-10 new cases per 1 million people worldwide, with variations in different regions and among different ethnic groups. Incidence is increased in patients of Ashkenazi Jewish descent and those of Mediterranean origin as high as 16 to 32 cases per million people. are proteins (desmogleins) which act as the glue that attaches adjacent skin cells, keeping the skin intact. When
autoantibodiesantibodies against the self attack desmogleins, the cells become separated from each other. The skin virtually becomes unglued. This causes burn-like lesions or blisters that do not heal naturally. In some cases, these blisters can cover a significant area of the skin.
The disease appears to affect people across racial and cultural lines, so it’s not possible to predict who may get
pemphigusPemphigus is a group of organ specific autoimmune diseases characterized by the production of autoantibodies to desmogleins. They are mucocutaneous blistering diseases that demonstrate a loss of cohesion of the epidermal cells (acantholysis) which result in the formation of clefts in the epidermis. Autoantibodies directed against adhesion molecules cause epidermal keratinocytes to separate, resulting in intraepidermal bullae. There are two histological types of pemphigus: deep (e.g., pemphigus vulgaris) and superficial (e.g., pemphigus foliaceus). They differ in the epidermal layers that are affected, in the clinical manifestations of the diseases, and in the associated immunologic abnormalities. Pemphigus has an incidence of 1-10 new cases per 1 million people worldwide, with variations in different regions and among different ethnic groups. Incidence is increased in patients of Ashkenazi Jewish descent and those of Mediterranean origin as high as 16 to 32 cases per million people.. Although fairly common in adults,
pemphigusPemphigus is a group of organ specific autoimmune diseases characterized by the production of autoantibodies to desmogleins. They are mucocutaneous blistering diseases that demonstrate a loss of cohesion of the epidermal cells (acantholysis) which result in the formation of clefts in the epidermis. Autoantibodies directed against adhesion molecules cause epidermal keratinocytes to separate, resulting in intraepidermal bullae. There are two histological types of pemphigus: deep (e.g., pemphigus vulgaris) and superficial (e.g., pemphigus foliaceus). They differ in the epidermal layers that are affected, in the clinical manifestations of the diseases, and in the associated immunologic abnormalities. Pemphigus has an incidence of 1-10 new cases per 1 million people worldwide, with variations in different regions and among different ethnic groups. Incidence is increased in patients of Ashkenazi Jewish descent and those of Mediterranean origin as high as 16 to 32 cases per million people. is rare in children. In children, it is most often caused by placental transmission of IgG
autoantibodiesantibodies against the self in utero from a woman who has
pemphigusPemphigus is a group of organ specific autoimmune diseases characterized by the production of autoantibodies to desmogleins. They are mucocutaneous blistering diseases that demonstrate a loss of cohesion of the epidermal cells (acantholysis) which result in the formation of clefts in the epidermis. Autoantibodies directed against adhesion molecules cause epidermal keratinocytes to separate, resulting in intraepidermal bullae. There are two histological types of pemphigus: deep (e.g., pemphigus vulgaris) and superficial (e.g., pemphigus foliaceus). They differ in the epidermal layers that are affected, in the clinical manifestations of the diseases, and in the associated immunologic abnormalities. Pemphigus has an incidence of 1-10 new cases per 1 million people worldwide, with variations in different regions and among different ethnic groups. Incidence is increased in patients of Ashkenazi Jewish descent and those of Mediterranean origin as high as 16 to 32 cases per million people..
If left untreated, the erosions and bullae of
pemphigusPemphigus is a group of organ specific autoimmune diseases characterized by the production of autoantibodies to desmogleins. They are mucocutaneous blistering diseases that demonstrate a loss of cohesion of the epidermal cells (acantholysis) which result in the formation of clefts in the epidermis. Autoantibodies directed against adhesion molecules cause epidermal keratinocytes to separate, resulting in intraepidermal bullae. There are two histological types of pemphigus: deep (e.g., pemphigus vulgaris) and superficial (e.g., pemphigus foliaceus). They differ in the epidermal layers that are affected, in the clinical manifestations of the diseases, and in the associated immunologic abnormalities. Pemphigus has an incidence of 1-10 new cases per 1 million people worldwide, with variations in different regions and among different ethnic groups. Incidence is increased in patients of Ashkenazi Jewish descent and those of Mediterranean origin as high as 16 to 32 cases per million people. vulgaris gradually spread, involving an increasing surface area, and can become complicated by severe infections and metabolic disturbances.
Before the advent of corticosteroids,
pemphigusPemphigus is a group of organ specific autoimmune diseases characterized by the production of autoantibodies to desmogleins. They are mucocutaneous blistering diseases that demonstrate a loss of cohesion of the epidermal cells (acantholysis) which result in the formation of clefts in the epidermis. Autoantibodies directed against adhesion molecules cause epidermal keratinocytes to separate, resulting in intraepidermal bullae. There are two histological types of pemphigus: deep (e.g., pemphigus vulgaris) and superficial (e.g., pemphigus foliaceus). They differ in the epidermal layers that are affected, in the clinical manifestations of the diseases, and in the associated immunologic abnormalities. Pemphigus has an incidence of 1-10 new cases per 1 million people worldwide, with variations in different regions and among different ethnic groups. Incidence is increased in patients of Ashkenazi Jewish descent and those of Mediterranean origin as high as 16 to 32 cases per million people. had a high fatality rate, with approximately 70+% of patients dying within a year (q, p).
With treatment, lesions can heal normally without scarring and the hyperpigmentation associated with
pemphigusPemphigus is a group of organ specific autoimmune diseases characterized by the production of autoantibodies to desmogleins. They are mucocutaneous blistering diseases that demonstrate a loss of cohesion of the epidermal cells (acantholysis) which result in the formation of clefts in the epidermis. Autoantibodies directed against adhesion molecules cause epidermal keratinocytes to separate, resulting in intraepidermal bullae. There are two histological types of pemphigus: deep (e.g., pemphigus vulgaris) and superficial (e.g., pemphigus foliaceus). They differ in the epidermal layers that are affected, in the clinical manifestations of the diseases, and in the associated immunologic abnormalities. Pemphigus has an incidence of 1-10 new cases per 1 million people worldwide, with variations in different regions and among different ethnic groups. Incidence is increased in patients of Ashkenazi Jewish descent and those of Mediterranean origin as high as 16 to 32 cases per million people. often resolves after several months. Most patients treated for
pemphigusPemphigus is a group of organ specific autoimmune diseases characterized by the production of autoantibodies to desmogleins. They are mucocutaneous blistering diseases that demonstrate a loss of cohesion of the epidermal cells (acantholysis) which result in the formation of clefts in the epidermis. Autoantibodies directed against adhesion molecules cause epidermal keratinocytes to separate, resulting in intraepidermal bullae. There are two histological types of pemphigus: deep (e.g., pemphigus vulgaris) and superficial (e.g., pemphigus foliaceus). They differ in the epidermal layers that are affected, in the clinical manifestations of the diseases, and in the associated immunologic abnormalities. Pemphigus has an incidence of 1-10 new cases per 1 million people worldwide, with variations in different regions and among different ethnic groups. Incidence is increased in patients of Ashkenazi Jewish descent and those of Mediterranean origin as high as 16 to 32 cases per million people. will enter a partial remission within 2 to 5 years. (In a longitudinal study of outcome in 40 patients with
pemphigusPemphigus is a group of organ specific autoimmune diseases characterized by the production of autoantibodies to desmogleins. They are mucocutaneous blistering diseases that demonstrate a loss of cohesion of the epidermal cells (acantholysis) which result in the formation of clefts in the epidermis. Autoantibodies directed against adhesion molecules cause epidermal keratinocytes to separate, resulting in intraepidermal bullae. There are two histological types of pemphigus: deep (e.g., pemphigus vulgaris) and superficial (e.g., pemphigus foliaceus). They differ in the epidermal layers that are affected, in the clinical manifestations of the diseases, and in the associated immunologic abnormalities. Pemphigus has an incidence of 1-10 new cases per 1 million people worldwide, with variations in different regions and among different ethnic groups. Incidence is increased in patients of Ashkenazi Jewish descent and those of Mediterranean origin as high as 16 to 32 cases per million people. vulgaris, 45% entered a complete and long-term remission after 5 years and 71% after 10 years.
PemphigusPemphigus is a group of organ specific autoimmune diseases characterized by the production of autoantibodies to desmogleins. They are mucocutaneous blistering diseases that demonstrate a loss of cohesion of the epidermal cells (acantholysis) which result in the formation of clefts in the epidermis. Autoantibodies directed against adhesion molecules cause epidermal keratinocytes to separate, resulting in intraepidermal bullae. There are two histological types of pemphigus: deep (e.g., pemphigus vulgaris) and superficial (e.g., pemphigus foliaceus). They differ in the epidermal layers that are affected, in the clinical manifestations of the diseases, and in the associated immunologic abnormalities. Pemphigus has an incidence of 1-10 new cases per 1 million people worldwide, with variations in different regions and among different ethnic groups. Incidence is increased in patients of Ashkenazi Jewish descent and those of Mediterranean origin as high as6 to 32 cases per million people. Vulgaris (PV)
- The term “vulgar” means “common,” and PV is the most commonly diagnosed form of pemphigusPemphigus is a group of organ specific autoimmune diseases characterized by the production of autoantibodies to desmogleins. They are mucocutaneous blistering diseases that demonstrate a loss of cohesion of the epidermal cells (acantholysis) which result in the formation of clefts in the epidermis. Autoantibodies directed against adhesion molecules cause epidermal keratinocytes to separate, resulting in intraepidermal bullae. There are two histological types of pemphigus: deep (e.g., pemphigus vulgaris) and superficial (e.g., pemphigus foliaceus). They differ in the epidermal layers that are affected, in the clinical manifestations of the diseases, and in the associated immunologic abnormalities. Pemphigus has an incidence of 1-10 new cases per 1 million people worldwide, with variations in different regions and among different ethnic groups. Incidence is increased in patients of Ashkenazi Jewish descent and those of Mediterranean origin as high as 16 to 32 cases per million people..
- Sores and blisters almost always start in the mouth. The blisters can go as far down as the vocal cords, but no further.
- The lesions are painful. Sometimes there is the effect that when just touching the skin can cause it to tear.
- PV does not cause permanent scaring unless there is infection associated with the sore.
- Today, with current therapies, the mortality rate is between 5% to 15%.
The information above was obtained from The International Pemphigus Pemphigoid Foundation. To learn more, visit
www.pemphigus.org