What is IgA Pemphigus?

What is IgA Pemphigus?

IgA pemphigus is a rare autoimmune blistering disease characterized by painful and pruritic vesiculopustular eruptions. These eruptions form as a result of circulating IgA antibodies against keratinocyte cell surface components responsible for cell to cell adherence.

What does Subcorneal mean?

Subcorneal pustular dermatosis (SPD) is a rare skin disease in which pus-filled pimples or blisters (pustules) form under the top (subcorneal) layer of the skin. It is most common in middle-aged adults (particularly women) but can develop in children.

What is Sneddon Wilkinson disease?

Subcorneal pustular dermatosis (SPD), also known as Sneddon-Wilkinson disease, is a rare neutrophilic dermatosis in which recurrent crops of sterile pustules appear in the most superficial (subcorneal) layers of the skin (picture 1A-B).

What is pemphigus vulgaris?

What is pemphigus vulgaris? Pemphigus is a rare group of autoimmune diseases. It causes blisters on the skin and mucous membranes throughout the body. It can affect the mouth, nose, throat, eyes, and genitals. Pemphigus vulgaris is the most common type of pemphigus.

Is pustule contagious?

The pus in pustules is caused by inflammation and is not contagious.

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.

Which is the best antibody panel for pemphigus?

Pemphigus Antibody Panel – Epithelial Cell Surface Antibodies and Desmoglein 1 and Desmoglein 3 Antibodies, IgG Copied! Recommendations when to order or not order the test. May include related or preferred tests. Use as preferred serum antibody panel for initial assessment and disease monitoring in IgG-variant pemphigus.

Which is the most common type of pemphigus?

Pemphigus vulgaris and pemphigus foliaceus are the most common types of IgG-variant pemphigus with blistering and erosive disease affecting skin and mucous membranes. Panel components include antibody testing for IgG epithelial cell surface and IgG desmoglein 1 and 3.

What kind of biopsy is needed for pemphigus vulgaris?

Testing should be clinically supported and/or correlated with concurrent Cutaneous Direct Immunofluorescence, Biopsy ( 0092572 ). Pemphigus vulgaris and pemphigus foliaceus are the most common types of IgG-variant pemphigus with blistering and erosive disease affecting skin and mucous membranes.