What is the pathogenesis of pemphigus vulgaris?
What is the pathogenesis of pemphigus vulgaris?
Pathophysiology. Pemphigus is an autoimmune disease caused by antibodies directed against both desmoglein 1 and desmoglein 3 present in desmosomes. Loss of desmosomes results in loss of cohesion between keratinocytes in the epidermis, and a disruption of the barrier function served by intact skin.
What pathological process is characteristic for pemphigus vulgaris?
The term pemphigus refers to a group of autoimmune blistering diseases of the skin and mucous membranes characterized histologically by intraepidermal blister and immunopathologically by the finding of in vivo bound and circulating immunoglobulin G (IgG) antibody directed against the cell surface of keratinocytes.
What is the difference between pemphigus vulgaris and bullous pemphigoid?
Pemphigus is a chronic and potentially fatal disease and patients should be counseled accordingly. Bullous pemphigoid is usually less severe and can resolve in 1 – 2 years.
How is pemphigus vulgaris causes?
Pemphigus vulgaris is not fully understood. Experts believe that it’s triggered when a person who has a genetic tendency to get this condition comes into contact with an environmental trigger, such as a chemical or a drug. In some cases, pemphigus vulgaris will go away once the trigger is removed.
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.
How is pemphigus vulgaris diagnosed?
The diagnosis of pemphigus vulgaris is confirmed by biopsy of lesional and surrounding (perilesional) normal skin. Immunofluorescence testing shows IgG autoantibodies against the keratinocyte’s cell surface.
How does pemphigus start?
Pemphigus vulgaris occurs when the immune system mistakenly makes antibodies against proteins in healthy skin and mucous membranes. The antibodies break down the bonds between the cells, and fluid collects between the layers of the skin. This leads to blisters and erosions on the skin.
What is the best treatment for pemphigus vulgaris?
Systemic corticosteroids remain the gold standard treatment for pemphigus vulgaris. Azathioprine and mycophenolate mofetil are the first line of steroid-sparing treatment. Rituximab is extremely effective in recalcitrant pemphigus, when other treatments fail to control the disease.
Can stress cause pemphigus vulgaris?
For those of you who have any one of the pemphigus/pemphigoid (P/P) related skin diseases, stress is the number one factor in flare-ups occurring.
Which layer does pemphigus vulgaris affect?
Now, all of the layers of the epidermis are rich in desmoglein 1 and 3, while mucosal cells predominantly have desmoglein 3. So, in individuals with IgG antibodies that bind to both desmoglein 1 and 3, a person develops mucocutaneous pemphigus vulgaris because it affects both the skin and the mucosa.
What is pemphigus Foliaceus in humans?
Pemphigus foliaceus is a rare autoimmune blistering disease which is characterised by superficial blisters, erosions and crusts on the skin. This type of pemphigus is less common than pemphigus vulgaris in Australia but in some parts of the world it is “endemic” and very common.
How serious is pemphigus?
Pemphigus can occur at any age, but it’s most often seen in people who are middle-aged or older. It tends to be a long-lasting (chronic) condition, and some types can be life-threatening without treatment. Treatment with medication usually controls it.
What is the difference between pemphigus and pemphigoid?
Another difference between pemphigus and pemphigoid is the type of blister each disease creates. A pemphigus blister is fragile, breaks easily, and leaves raw skin. A pemphigoid blister is tight and does not break easily, but it does cause pain and itchiness.
What causes pemphigus foliaceus?
Pemphigus foliaceus is sometimes provoked by sun exposure. Endemic pemphigus foliaceus occurs in South America, where it is commonly known as Fogo selvagem . It appears to be set off by a virus transmitted by an insect bite.
What is the pathophysiology of pemphigus herpetiformis?
Pathophysiology Pemphigus Herpetiformis is an IGg mediated autoantibodies that affect the epidermal layer of the skin.
What is pemphigus and pemphigoid?
Pemphigus and pemphigoid are two similar but different diseases. They are both diseases of the immune system, attacking different cells, and both cause blisters, though they differ in the types of blisters they cause as well as their locations.