Back

PEMPHIGUS VULGARIS

Note for Pet Owners:

This information is provided by Provet for educational purposes only.

You should seek the advice of your veterinarian if your pet is ill as only he or she can correctly advise on the diagnosis and recommend the treatment that is most appropriate for your pet.

Topics on this Page:


Description
Pemphigus vulgaris is a rare automimmune skin disease which is seen in dogs and cats. The disease causes ulceration and vesicles to occur in the skin - particularly at the muco-cutaneous junctions eg the lips, the nail beds, and in the mouth  

Without treatment the disease can be fatal.


Cause
The cause is an autoimmune disorder
in which an IgG autoantibody binds to intracellular adhesion molecules causing a loss of cohesion between keratinocytes resulting in acantholysis, vesicle formation and eventually ulceration.


Breed Occurrence
Pemphigus vulgaris is the commonest autoimmune skin disease in humans, but it is rare in cats and dogs. 

Most authors now agree that there is no breed, sex or age predilection for this disease, but some breeds are mentioned by other authors. eg German Shepherd Dog, 


Signs
Vesicles, ulceration and crusting can affect the whole body, but especially affects the muco-cutaneous junctions and oral mucosa. Secondary infection of the open ulcers can occur. The onset of the disease can be sudden (acute) or gradual over many weeks (chronic) Affected animals are usually depressed and inappetent with a high body temperature.


Complications
Secondary infections


Diagnosis
Diagnosis is confirmed on histopathological examination of biopsies. The lesions typically reveal acantholysis with an intraepidermal suprabasilar cleft. Direct and Indirect immunofluoresence show positive staining of intracellular particles.

Differential diagnosis

  • Candidasis
  • Systemic Lupus Erythematosus
  • Bullous Pemphigoid
  • Drug eruption
  • Skin cancer

Treatment

  • Prednisolone at dose - 2-4 mg/kg body weight every 12 hours by mouth .
  • If corticosteroids alone do not induce remission after 14 days a cytotoxic drug** should be added to the regimen, the following have been used successfully :
    • Azothiaprine* 2 mg/kg body weight (50mg/m2 ) on alternate days (Contraindicated for use in cats)
    • Cyclophosphamide 2 mg/kg body weight (50mg/m2) for 4 days per week
    • Aurothioglucose *1mg/kg body weight by intramuscular injection once weekly
    • Auranofin* 0.05-0.2 mg/kg body weight by mouth every 12 hours
    • Chlorambucil (preferred for cats) - 2-5mg/m2 every 1-2 days by mouth

* Bone marrow suppression is common with these drugs and blood cell status should be monitored regularly.

** Extreme care is needed when handling these drugs

With all the above therapeutic agents, the dose rate is gradually reduced to the minimum maintenance dose (eg alternate day therapy) once the animal is in remission.

 


Prognosis
Poor for the most severely affected animals. Euthanasia is often performed.


Long term problems
All the therapeutic agents, at the doses required to control pemphigus vulgaris have serious side-effects which can in themselves be life-threatening.

 

Updated October 2013

 
d>