IgA pemphigus is a subtype of
pemphigus
Pemphigus ( or ) is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes.
The name is derived from the Greek root ''pemphix'', meaning "blister".
In pemphigus, autoantibody, autoantibodies form against de ...
with two distinct forms:
* ''Subcorneal pustular dermatosis'' (also known as Sneddon–Wilkinson disease and pustulosis subcornealis) is skin condition that is a rare, chronic, recurrent, pustular eruption characterized histopathologically by subcorneal pustules that contain abundant neutrophils.
[James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. .] This is distinct from and not to be confused with
subcorneal pustular dermatosis type of IgA pemphigus.
Sneddon's syndrome, also known as Ehrmann-Sneddon syndrome, is also a different syndrome.
* ''Intraepidermal neutrophilic IgA dermatosis'' is characterized
histologically
Histology,
also known as microscopic anatomy or microanatomy, is the branch of biology that studies the microscopic anatomy of biological tissue (biology), tissues. Histology is the microscopic counterpart to gross anatomy, which looks at large ...
by
intraepidermal bullae with
neutrophils
Neutrophils are a type of phagocytic white blood cell and part of innate immunity. More specifically, they form the most abundant type of granulocytes and make up 40% to 70% of all white blood cells in humans. Their functions vary in different ...
, some
eosinophils
Eosinophils, sometimes called eosinophiles or, less commonly, acidophils, are a variety of white blood cells and one of the immune system components responsible for combating multicellular parasites and certain infections in vertebrates. Along wi ...
, and
acantholysis.
History
Early descriptions were made by
Darrell Wilkinson, a British dermatologist.
See also
*
Pemphigus
Pemphigus ( or ) is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes.
The name is derived from the Greek root ''pemphix'', meaning "blister".
In pemphigus, autoantibody, autoantibodies form against de ...
*
List of target antigens in pemphigus
*
List of conditions caused by problems with junctional proteins
*
References
External links
Chronic blistering cutaneous conditions
{{Vesiculobullous disease