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 "pustule".
In pemphigus, autoantibodies form against desmoglein, whi ...
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
IgA pemphigus is a subtype of pemphigus 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 ...
.
Sneddon's syndrome, also known as Ehrmann-Sneddon syndrome, is also a different syndrome.
* ''Intraepidermal neutrophilic IgA dermatosis'' is characterized
histologically by
intraepidermal bullae with
neutrophils
Neutrophils (also known as neutrocytes or heterophils) are the most abundant type of granulocytes and make up 40% to 70% of all white blood cells in humans. They form an essential part of the innate immune system, with their functions varying i ...
, some
eosinophils
Eosinophils, sometimes called eosinophiles or, less commonly, acidophils, are a variety of white blood cells (WBCs) and one of the immune system components responsible for combating multicellular parasites and certain infections in vertebrates. A ...
, and
acantholysis
Acantholysis is the loss of intercellular connections, such as desmosomes, resulting in loss of cohesion between keratinocytes,Kumar, Vinay; Fausto, Nelso; Abbas, Abul (2004) ''Robbins & Cotran Pathologic Basis of Disease'' (7th ed.). Saunders. ...
.
History
Early descriptions were made by
Darrell Wilkinson
Peter Edward Darrell Sheldon Wilkinson (7 August 1919 – 17 August 2009) was a leading figure in dermatology who was consultant at Aylesbury and High Wycombe, co-edited the first edition of Rook's 1968 '' Textbook of Dermatology'' and found ...
, 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 "pustule".
In pemphigus, autoantibodies form against desmoglein, whi ...
*
List of cutaneous conditions
Many skin conditions affect the human integumentary system—the organ system covering the entire surface of the body and composed of skin, hair, nails, and related muscle and glands. The major function of this system is as a barrier agai ...
*
List of conditions caused by problems with junctional proteins
*
References
External links
Chronic blistering cutaneous conditions
{{Vesiculobullous disease