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Pemphigus vulgaris is a rare chronic blistering skin disease and the most common form of pemphigus.Pemphigus was derived from the Greek word pemphix, meaning blister. [1] It is classified as a type II hypersensitivity reaction in which antibodies are formed against desmosomes, components of the skin that function to keep certain layers of skin bound to each other.
Circulating auto-antibodies in the human body can target normal parts of the skin leading to disease. This is a list of antigens in the skin that may become targets of circulating auto-antibodies leading to the various types of pemphigus .
Pemphigus is a group of autoimmune blistering diseases that may be classified into these types: [14] Pemphigus vulgaris; Pemphigus vegetans; Pemphigus vegetans of Hallopeau; Pemphigus vegetans of Neumann; Pemphigus foliaceus, of which there several forms: Pemphigus erythematosus or Senear–Usher Syndrome
This article provides a list of autoimmune diseases. These conditions, where the body's immune system mistakenly attacks its own cells, affect a range of organs and systems within the body. Each disorder is listed with the primary organ or body part that it affects and the associated autoantibodies that are typically found in people diagnosed ...
Mycophenolate is increasingly utilized as a steroid sparing treatment in autoimmune diseases and similar immune-mediated disorders including Behçet's disease, pemphigus vulgaris, immunoglobulin A nephropathy, small vessel vasculitides, and psoriasis. [21] It is also used for retroperitoneal fibrosis along with a number of other medications. [22]
The cause of the disease is a haploinsufficiency of the enzyme ATP2C1; [7] the ATP2C1 gene is located on chromosome 3, which encodes the protein hSPCA1.A mutation on one copy of the gene causes only half of this necessary protein to be made and the cells of the skin do not adhere together properly due to malformation of intercellular desmosomes, causing acantholysis, blisters and rashes.
It is also associated with pemphigus vulgaris and pemphigus foliaceus. [6] [7] It is useful in differentiating between the diagnosis of pemphigus vulgaris or mucous membrane pemphigoid (where the sign is present) and bullous pemphigoid (where it is absent). The Nikolsky sign is dislodgement of intact superficial epidermis by a shearing force ...
Bullous pemphigoid (a type of pemphigoid) is an autoimmune pruritic skin disease that typically occurs in people aged over 60, that may involve the formation of blisters in the space between the epidermal and dermal skin layers.