Immunosuppression is the main foundation therapy for dogs diagnosed with pemphigus foliaceus. Corticosteroids serve as the
foundation treatment, tend to provide clinical remission, but may produce undesirable side effects. Initially, the dosage
of prednisone for the treatment of pemphigus foliaceus ranges from 2-6 mg/kg daily. My experience with prednisone in dogs,
is the occurrence of unacceptable polydypsia and polyuria as well as incontinence occurs more frequently than with methylprednisolone.
Methylprednisolone is 20 percent more potent than prednisone or prednisolone, so the dosage is a bit lower. Triamcinolone
(available in 0.5 mg and 1.5 mg tablets) can be most useful in cats and should be reserved for severe, refractory cases in
the dog. Triamcinolone is about 10 times more potent than prednisone per milligram. Dexamethasone is safe only for cats and
has a similar potency as triamcinolone.
In most cases of pemphigus foliaceus, the disease tends to be chronic. As a result, I recommend the addition of azathioprine
(chlorambucil in the cat) as an adjunct to corticosteroid therapy. The goal would be to taper off corticosteroid therapy and
use azathioprine long-term. The dosage of azathioprine is 1-2 mg/kg/day daily for one to two weeks, then reduced to every
other day or every third day. Monitoring the results of complete blood counts (CBC) as well as liver enzymes is important,
since bone suppression and hepatotoxicity are rare but potentially serious side effects. A CBC is recommended two weeks after
initiation of therapy, then every two to three months. Blood chemistries, including measurement of liver enzymes, should be
performed every three to six months.
Photo 4: Results of the skin histopathology show broad, superficial subcorneal pustules containing varying degrees of acantholytic
Alternatives to corticosteroids and azathioprine for dogs include cyclosporine (10 mg/kg/day) as a single agent. Cyclosporine
inhibits activated T-cells and some cytokines. This drug can cause gastritis (vomiting), diarrhea and renal failure (rare
and difficult to prove). Famotidine administered 30 minutes prior to cyclosporine can reduce or eliminate vomiting and nausea.
Viral papilloma formation, hypertrichosis, gingival hyperplasia and bizarre skin eruptions have also been experienced in rare
Luflunomide, a strong immunosuppressant, now available as generic, has been used rarely in dogs for treatment of auto-immune
disease. It inhibits T and B cells, antibody synthesis and antagonizes several cytokines. The dosage is 2-6 mg/kg/day. Trough
levels should be achieved at around 30ug/ml.
Cellcept (mycophenolate mofetil) is a relatively new immunosuppressant that has been used rarely in dogs for the treatment
of auto-immune diseases. This drug has potent cytostatic effects on T and B cells. Cellcept also suppresses antibody formation.
The dosage is about 20 mg/kg BID. Diarrhea is the main side effect and is dose dependent.
The exact pathomechanism of canine pemphigus foliaceus is not known but it is considered to be an auto-immune disease involving
the production of auto-antibodies (Immunoglobulin G, rarely Complement 3). The autoantigen is a component of the desmosome
(desmoglein) that is considered a normal structure and a part of the intercellular "bridging" that allows epidermal cells
to remain connected. It is part of the cytoskeleton and allows the epidermis to be part of the skin tissue. As a result of
this antibody deposition, the epidermal cells (keratinocytes) lose their cohesion and fall apart (acantholysis). In a very
short time after this acantholysis (blister formation), the canine immune system recruits neutrophils that quickly migrate
into the inter-keratinocyte space, thus producing the classic pustule seen clinically, as well as histologically. This is
what occurs in humans, except for the influx of neutrophils.
There has been speculation that genetic factors play a role in humans, since certain genetic markers have been discovered
that link certain individuals to pemphigus vulgaris (more severe form of pemphigus that involves mucous membranes). Although
this has not been demonstrated or proven in dogs, a marked breed predilection occurs. Akitas, Chow Chows, Dachshunds, the
Finnish spitz, Bearded Collies and others are considered predisposed. In people, there is an endemic form of pemphigus foliaceus
reported in Brazil and is called "fogo selvagem" or "wild fire." Wild fire refers to the burning or even stinging sensation
that some patients experienced when exposed to sunlight. Epidemiologists speculated a black fly served as a vector. This was
believed to be true since most people who developed lesions were exposed to black fly bites, while others who were not exposed
did not develop lesions. Some human patients with pemphigus foliaceus may develop blisters in uninvolved skin. UV-B may be
the wavelength involved and may also be implicated in cases of experimentally-induced blisters in humans exposed to artificial
Other factors that have been implicated in the cause of pemphigus foliaceus in humans include drugs and neoplasia. The most
common drugs implicated in human pemphigus foliaceus include penicillamine and penicillin type antibiotics. In people, paraneoplastic
pemphigus foliaceus has been reported to occur in association with lympho-proliferative neoplasia. In the dog, there have
been two reports of paraneoplastic pemphigus foliaceus and have been related to thymic lymphoma and splenic sarcoma. There
has been some association in cats with the administration of methimazole and the onset of pemphigus foliaceus.
In conclusion, facial pemphigus foliaceus has a slightly different clinical (facially confined) distribution, deeper histologic
changes (follicular acantholysis) and can be refractory to some treatments. Knowledge about the known immunopathogenesis,
newer drugs and their mechanisms of action, and awareness of less expensive sources of certain drugs may allow successful
Dr. Vitale received his veterinary degree from Mississippi State University, College of Veterinary Medicine. He completed
a residency in veterinary dermatology at the University of California, Davis and is a diplomate of the American College of
Veterinary Dermatology. He is a clinical instructor/lecturer at UC-Davis and a staff dermatologist at East Bay Veterinary
Specialists (formerly Encina Veterinary Hospital), Bay Area Veterinary Specialists and San Francisco Veterinary Specialists.