IgA pemphigus

Medical condition
IgA pemphigus
Schematic of immunoglobulin A dimer showing H-chain (blue), L-chain (red), J-chain (magenta) and secretory component (yellow).
SpecialtyDermatology

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.[2][3]: 203  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.[4]
  • Intraepidermal neutrophilic IgA dermatosis is characterized histologically by intraepidermal bullae with neutrophils, some eosinophils, and acantholysis.[3]: 465 

History

Early descriptions were made by Darrell Wilkinson, a British dermatologist.[5]

See also

References

  1. ^ RESERVED, INSERM US14 -- ALL RIGHTS. "Orphanet: Subcorneal pustular dermatosis". www.orpha.net. Retrieved 8 March 2017.{{cite web}}: CS1 maint: numeric names: authors list (link)
  2. ^ Irwin M. Freedberg; et al., eds. (2003). Fitzpatrick's dermatology in general medicine (6th ed.). New York, NY [u.a.]: McGraw-Hill. p. 625. ISBN 978-0-07-138076-8.
  3. ^ a b James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
  4. ^ Berlit, Peter. "Sneddon's Syndrome". Orphanet.
  5. ^ "Munks Roll Details for Peter Edward Darrell Sheldon Wilkinson". munksroll.rcplondon.ac.uk. Retrieved 2017-11-10.

External links

Classification
D
External resources
  • eMedicine: article/1063776
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Acantholysis
(epidermis)
Pemphigus
Other
Pemphigoid
(dermis)
IgG:
IgA:
Other bullous
In diseases
classified elsewhere