ISSN 2398-2969      

Skin: pemphigus vulgaris

icanis

Introduction

  • Rare - one of the rarest autoimmune diseases in veterinary practice. 
  • Most severe of the pemphigus group; only one showing suprabasilar intra-epidermal vesicles.
  • Cause: autoantibody against intercellular cement (desmoglein III) → loss of epidermal adhesion → acantholysis.
  • Signs: autoimmune vesiculobullous, ulcerative condition of the skin, oral cavity and mucocutaneous areas.
  • Diagnosis: history, clinical signs, histopathology.
  • Treatment: immunosuppressive chemotherapy.
  • Prognosis: poor, needs lifelong, aggressive therapy with high risk of side effects. 

Referral of suspected cases to a veterinary dermatologist is recommended. 

Print off the owner factsheet Pemphigus to give to your client.

Pathogenesis

Etiology

  • Unknown initiation of autoantibody.
  • Genetic factors important → breed and familial predispositions.
  • Drug-induced, UV light and stress.
  • Prior chronic skin disease.
  • Possible virus with insect vector (human form in South America has suspected insect vector).

Predisposing factors

General

  • Prior drug treatment.
  • Prior skin disease.

Pathophysiology

  • Binding of pemphigus antibody to antigen on cell surface of keratinocyte → incorporated into keratinocyte lysosome.
  • Activation and release of proteolytic enzyme → diffuses into extracellular space → converts plasminogen to plasmin → hydrolysis of adhesion molecules → acantholysis → blister formation within epidermis.

Timecourse

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

Diagnosis

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Treatment

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Prevention

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Outcomes

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Further Reading

Publications

Refereed papers

  • Recent references from PubMed and VetMedResource.
  • Martinez N, McDonald B, Crowley A (2022) A case report of the beneficial effect of oclacitinib in a dog with pemphigus vulgaris. Vet Derm 33 (3), 237 PubMed.
  • Tham H L, Linder K E, Olivry T (2020) Deep pemphigus (pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus) in dogs, cats and horses: a comprehensive review. BMC Vet Res16, 457 PubMed.
  • White S D (1994) Diseases of the nasal planum. Vet Clin North Am 24 (5), 887-895 PubMed.
  • Johnson B W & Campbell K L (1989) Dermatoses of the canine eyelid. Comp Cont Ed Pract Vet 11 (4), 385-394 VetMedResource.
  • Carlotti D (1989) Proceedings of 1987 symposium on skin disease at Lanwade Hall, Newmarket. JSAP 30, 223-227.

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