Felis ISSN 2398-2950

Skin: pemphigus vulgaris

Contributor(s): Prof Douglass Macintire, David Scarff, Richard Squires

Introduction

  • Most severe of the pemphigus group of conditions; showing suprabasilar vesicles and clefts.
  • Extremely rare auto-immune disease of skin, oral cavity and mucocutaneous areas; but diagnosed second most frequently of the pemphigus group. (Pemphigus foliaceus Skin: pemphigus foliaceus is the most common.)
  • Cause: auto-antibody against keratinocyte intercellular adhesion molecules.
  • Diagnosis: histopathology.
  • Treatment: immunosuppressive therapy.
  • Prognosis: poor.
    Print off the owner factsheet on Pemphigus  Pemphigus  to give to your client.

Pathogenesis

Etiology

  • Unknown initiation of auto-antibody.

Predisposing factors

General

  • In man and dogs predisposing factors may be important:
    • Drug-induced, ultraviolet light and stress.
    • Prior chronic skin disease.
    • Possible virus with insect vector.
  • The importance of these has not been established in the cat.

Pathophysiology

  • Damage to keratinocyte adhesion   →   blister formation   →   ulcerated with minor trauma.
  • Binding of pemphigus antibody to adhesion molecule on cell surface of keratinocyte   →   incorporated into keratinocyte lysosome.
  • Activation and release of proteolytic enzyme   →   extracellular space   →   converts plasminogen to plasmin   →   hydrolysis of adhesion molecules   →   acantholysis   →   blister formation within epidermis.
  • May be complement activation and chemoattraction of inflammatory cells (pustule formation).

Timecourse

  • Rapid onset of symptoms leads to death if no treatment.

Diagnosis

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Treatment

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Outcomes

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Further Reading

Publications

Refereed papers

  • Recent references from PubMed and VetMedResource.
  • Scott D W, Walton D K, Slater M R et al (1987) Immune-mediated dermatoses in domestic animals: ten years after. I. Comp Cont Educ Pract Vet (4), 424-435 VetMedResource.
  • Manning T O, Scott D W, Smith C A et al (1982) Pemphigus diseases in the feline; seven cases reports and discussion. JAAHA 18 (3), 433-443 VetMedResource.


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