Subcorneal pustular dermatosis
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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. 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 which studies the microscopic anatomy of biological tissues. Histology is the microscopic counterpart to gross anatomy, which looks at larger structures vis ...
by intraepidermal bullae with neutrophils, 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, 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 *
List of conditions caused by problems with junctional proteins Mutations of proteins that hold the cells of the skin together can cause disease. Autoantibodies against proteins that hold the cells of the skin together can also cause disease. See also * List of keratins expressed in the human integume ...
*
List of immunofluorescence findings for autoimmune bullous conditions Several cutaneous conditions can be diagnosed with the aid of immunofluorescence studies. Cutaneous conditions with positive direct or indirect immunofluorescence when using salt-split skin include: For several subtypes of pemphigus a variety ...


References


External links

Chronic blistering cutaneous conditions {{Vesiculobullous disease