IgA pemphigus

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 skin disease that causes blisters and pustules. Skin lesions often begin as firm blisters filled with clear fluid and later change into pus-filled lesions as inflammatory cells accumulate. [1]Some patients develop red patches of skin, while others do not, and in some cases the lesions may appear in a herpetiform (clustered) pattern. [2] Pruritus is reported in approximately half of patients with IgA pemphigus. [3]

IgA pemphigus is characterized by IgA autoantibodies against keratinocyte cell surface antigens, producing epidermal staining on direct and indirect immunofluorescence. [4]

IgA pemphigus is a subtype of pemphigus with two distinct forms:

  • Subcorneal pustular dermatosis (also known as Sneddon–Wilkinson disease and pustulosis subcornealis)[5] is skin condition that is a rare, chronic, recurrent, pustular eruption characterized histopathologically by subcorneal pustules that contain abundant neutrophils.[6][7]: 203  Clinically, in the IgA pemphigus–associated SPD type, patients may present with an annular pattern of red pustules with central crusting.[8] 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.[9]
  • Intraepidermal neutrophilic IgA dermatosis is characterized histologically by intraepidermal bullae with neutrophils, some eosinophils, and acantholysis.[7]: 465  Clinically, the pustules in the intraepidermal neutrophilic subtype may show a characteristic sunflower-like arrangement.[8]

History

The condition was first described by Ian Sneddon, a British physician, and Darrell Wilkinson, a British dermatologist.[10]

Epidemiology and Clinical Presentation

  • The majority of patients exhibited vesicles (80.8%), pustules (75.0%), and circinate plaques (63.6%).[11]

Treatment

  • The most commonly administered treatments were oral dapsone and corticosteroids.[12]

See also

References

  1. ^ Tsuruta, Daisuke; Ishii, Norito; Hamada, Takahiro; Ohyama, Bungo; Fukuda, Shunpei; Koga, Hiroshi; Imamura, Kazuko; Kobayashi, Hiromi; Karashima, Tadashi; Nakama, Takekuni; Dainichi, Teruki; Hashimoto, Takashi (2011). "IgA pemphigus". Clinics in Dermatology. 29 (4): 437–442. doi:10.1016/j.clindermatol.2011.01.014. ISSN 1879-1131. PMID 21679872.
  2. ^ Tsuruta, Daisuke; Ishii, Norito; Hamada, Takahiro; Ohyama, Bungo; Fukuda, Shunpei; Koga, Hiroshi; Imamura, Kazuko; Kobayashi, Hiromi; Karashima, Tadashi; Nakama, Takekuni; Dainichi, Teruki; Hashimoto, Takashi (2011). "IgA pemphigus". Clinics in Dermatology. 29 (4): 437–442. doi:10.1016/j.clindermatol.2011.01.014. ISSN 1879-1131. PMID 21679872.
  3. ^ Tsuruta, Daisuke; Ishii, Norito; Hamada, Takahiro; Ohyama, Bungo; Fukuda, Shunpei; Koga, Hiroshi; Imamura, Kazuko; Kobayashi, Hiromi; Karashima, Tadashi; Nakama, Takekuni; Dainichi, Teruki; Hashimoto, Takashi (2011). "IgA pemphigus". Clinics in Dermatology. 29 (4): 437–442. doi:10.1016/j.clindermatol.2011.01.014. ISSN 1879-1131. PMID 21679872.
  4. ^ Kridin, Khalaf; Patel, Payal M.; Jones, Virginia A.; Cordova, Adriana; Amber, Kyle T. (June 2020). "IgA pemphigus: A systematic review". Journal of the American Academy of Dermatology. 82 (6): 1386–1392. doi:10.1016/j.jaad.2019.11.059. ISSN 1097-6787. PMID 31812619.
  5. ^ 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)
  6. ^ 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.
  7. ^ a b James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
  8. ^ a b Khan Mohammad Beigi, Pooya (2018). A Clinician's Guide to Pemphigus Vulgaris. doi:10.1007/978-3-319-67759-0.
  9. ^ Berlit, Peter. "Sneddon's Syndrome". Orphanet.
  10. ^ Watts, Paula Jean; Khachemoune, Amor (December 2016). "Subcorneal Pustular Dermatosis: A Review of 30 Years of Progress". American Journal of Clinical Dermatology. 17 (6): 653–671. doi:10.1007/s40257-016-0202-8. ISSN 1179-1888. PMID 27349653.
  11. ^ Kridin, Khalaf; Patel, Payal M.; Jones, Virginia A.; Cordova, Adriana; Amber, Kyle T. (June 2020). "IgA pemphigus: A systematic review". Journal of the American Academy of Dermatology. 82 (6): 1386–1392. doi:10.1016/j.jaad.2019.11.059. ISSN 1097-6787. PMID 31812619.
  12. ^ Kridin, Khalaf; Patel, Payal M.; Jones, Virginia A.; Cordova, Adriana; Amber, Kyle T. (June 2020). "IgA pemphigus: A systematic review". Journal of the American Academy of Dermatology. 82 (6): 1386–1392. doi:10.1016/j.jaad.2019.11.059. ISSN 1097-6787. PMID 31812619.
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