Pemphigus foliaceus

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Pemphigus foliaceus
Classification and external resources
ICD-10 L10.2
DiseasesDB 31179

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Kiran Singh, M.D. [2]

Overview

Pemphigus foliaceus is an autoimmune blistering disease of the skin with characteristic lesions that are scaly, crusted erosions, often on an erythematous base.[1] Mucosal involvement is absent even with widespread disease.[2]:423

If there is an autoimmune IgG buildup in the epidermis, then nearly almost all of the antibodies are aimed against desmoglein 1. The effect of the antibodies and the immunological pathway is most likely either of three mechanisms:

  • Steric hindrance of the desmoglein 1: The antibody caps off the site for intracellular binding to another keratinocyte.
  • Activation of an endocytic pathway: The antibody activates a pathway which causes an internalization of desmogleïn 1, which in turn causes a loss of adhesion.
  • Disruption of function: In this case, the antibody blocks the desmoglein 1 from being formed into a desmosome. This in turn causes a loss of adhesion with acantholysis as a result.

Distribution

Pemphigus is endemic in the rural areas of Brazil, especially along inland riverbeds.[1]

History

Pierre Louis Alphee Cazenave first described the disease in 1844.[3]

Diagnosis

Physical Examination

Skin

Face
Trunk
Extremity
Pemphigus foliaceus-localized form
Face
Trunk

See also

References

  1. ↑ 1.0 1.1 Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). Page 558-562. McGraw-Hill. ISBN 0-07-138076-0.
  2. ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
  3. ↑ Medscape Reference
  4. ↑ 4.00 4.01 4.02 4.03 4.04 4.05 4.06 4.07 4.08 4.09 4.10 4.11 4.12 4.13 4.14 4.15 4.16 4.17 4.18 4.19 4.20 4.21 4.22 4.23 4.24 4.25 4.26 4.27 4.28 4.29 4.30 4.31 4.32 4.33 4.34 4.35 4.36 4.37 4.38 4.39 4.40 4.41 4.42 4.43 4.44 4.45 4.46 4.47 4.48 4.49 "Dermatology Atlas".

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