IgA pemphigus (or immunoglobulin A pemphigus) is an autoimmune blistering disorder. It is also called intercellular IgA dermatosis among other names. IgA pemphigus has two major subtypes: Deposition of intracellular IgA against the glycoprotein desmocollin-1 seen predominantly in the upper epidermis. Intraepidermal neutrophilic (IEN) type.

2024年5月1日 · IgA pemphigus is a rare autoimmune blistering disease characterized by painful and pruritic vesiculopustular eruptions resulting from IgA antibodies targeting keratinocyte cell surface components involved in cell-to-cell adherence. Two distinct forms of IgA pemphigus exist, both clinically similar but with distinct autoantibody target proteins.

IgA pemphigus, also known as intercellular IgA dermatosis, among other names, is a rare and poorly understood epidermal autoimmune blistering disease (AIBD). 1 The defining feature of this disease is the presence of IgA anti-keratinocyte cell surface autoantibodies, labeling the epidermal human skin by direct immunofluorescence (DIF) and ...

IgA pemphigus is a subtype of pemphigus with two distinct forms: Subcorneal pustular dermatosis (also known as Sneddon–Wilkinson disease and pustulosis subcornealis) [1] is skin condition that is a rare, chronic, recurrent, pustular eruption characterized histopathologically by subcorneal pustules that contain abundant neutrophils.

2024年5月1日 · Immunoglobulin A (IgA) pemphigus, a rare autoimmune blistering disease, manifests as painful and pruritic vesiculopustular eruptions. These eruptions occur due to circulating IgA antibodies targeting keratinocyte cell surface components involved in …

IgA pemphigus was first described by Wallach, Foldes, and Cottenot in 1982 under the name subcorneal pustular dermatosis and monoclonal IgA. 47 It is a group of autoimmune intraepidermal blistering diseases presenting with a vesiculopustular eruption, neutrophil infiltration, acantholysis and tissue-bound and circulating IgA antibodies ...

Immunoglobulin A (IgA) pemphigus is a group of newly characterized immune-mediated intraepidermal blistering skin diseases. Unlike typical immunoglobulin G (IgG)–mediated pemphigus,...

Objective: To synthesize existing data on the epidemiologic, clinical, histologic, and immunologic features of IgA pemphigus. Methods: We performed a systematic review using MEDLINE, Embase, and Web of Science databases. Case reports and …

The term intercellular IgA dermatosis (IAD) (also frequently called IgA pemphigus) refers to a group of AIBDs of skin and mucous membranes. IAD is characterized clinically by pustular skin lesions, histopathologically by intraepidermal neutrophilic pustules, and immunopathologically by in vivo bound and circulating IgA antibodies directed ...

IgA pemphigus is characterized by “pimples” or blisters. The vesicles and pustules are usually, but not always, accompanied by patches of redness. A herpes, ring-shaped, or circular pattern may be present. The trunk and nearby extremities are common sites for IgA pemphigus.