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Patience Required with Pemphigus Foliaceus

Posted Aug 14 2013 7:00am
Dr. Coates is a veterinarian based in the other “Sunshine State” – that's Colorado to the rest of you – where she lives and plays with a varied range of animals. She shares her professional and personal experiences, Monday through Friday, here on petMD's blog, the Fully Vetted. Log in for your daily dose of her insight and wisdom. < Previous Post Next Post > Aug 14, 2013 Patience Required with Pemphigus Foliaceus by Dr. Jennifer Coates, DVM     Share       Save to mypetMDI enjoy complicated dermatology cases, but many veterinarians find them unbearably frustrating. The reason behind this is simple. Owners want quick answers and permanent solutions, particularly when it comes to problems affecting the skin, and veterinarians don’t like to disappoint. Clients seem more willing to understand the need for a methodical work up when pets are “sick,” a description that sometimes doesn’t seem to apply when dealing with dermatological lesions.
 
Pemphigus foliaceus is a perfect example of this type of challenge. The disease often starts with pimple-like lesions around the head (the inner surface of the ear flap is predisposed) and feet. Because the pimples quickly rupture, the crusts that are left over are what tend to be observed by owners. Dogs and cats are generally not itchy, unless they develop a secondary skin infection, and feel well otherwise. Some pets present with skin lesions across most of their bodies, either because of owner inattention or a more aggressive variant of the disease.
 
Keep in mind that innumerable skin diseases cause similar symptoms, many of which are more common than pemphigus, so the veterinarian’s first job is to start winnowing down the list. I do this by running a skin scraping for mites, a fungal culture for ringworm, and skin cytology for infections. At this point we may get sidetracked for a few weeks. If I find an infection I’ll treat it, but if it’s being caused by pemphigus the lesions will only partially resolve. I may also put the dog or cat on a broad spectrum parasiticide like selamectin to rule out some of the external parasites that can be hard to diagnose, but this won’t have any effect on a case of pemphigus. Finally, fungal cultures take a few weeks to complete, and I try to wait on those results before proceeding.
 
By this point we’re usually 2-3 weeks out from our first appointment. During the recheck, all but the most understanding of clients are a little frustrated. They’ve spent a significant amount of time and money on their pets with little to show for it. Hopefully I’ve done a good job preparing them for the complexities of dermatology and they’re eager to move on to the next diagnostic step – a general health work-up including blood work and a urinalysis followed by skin biopsies.
 
The lab work is needed to rule out systemic illnesses. With pemphigus, the results are usually unremarkable but still serve the purpose of preparing for the safe, long-term use of medications to control the disease. The only way to definitively diagnose pemphigus is to send off multiple skin biopsies for review by a pathologist. When they see a characteristic change in a particular layer of skin, they can then make the call.
 
Pemphigus foliaceus is an autoimmune disease that is often worsened by exposure to sunlight. It is caused by the body attacking cells called desmosomes that connect other skin cells called keratinocytes. Treatment involves the use of immunosuppressives – usually steroids alone or in combination with other drugs like cyclosporine or azathioprine depending on the pet’s response. Pemphigus may be instigated by the use of certain types of medications, but the cause of most cases goes undiagnosed and is suspected to have genetic underpinnings.
 
Some patients may eventually be able to be weaned off immunosuppressive medications, but many dogs and cats require lifelong therapy to control the disease. The goal is to find the lowest doses of the drugs with the least side effects for that individual, a process that requires good communication between owner and veterinarian, quick response to flare-ups, regular rechecks, and continued large doses of patience.
 
 

Dr. Jennifer Coates
 
 
Image: Little Moon / Shutterstock
 
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Candi Costigan 08/14/2013 08:19am THANK YOU SOOOO MUCH!! I have been taking my cat to the same Vet for 5 years trying to figure out what those "little bumps" were.
Depo shots seem to be the thing that helps and we have to go in for a shot once every 3 months.

Reply to this comment Report abuse 10 oh holland 08/14/2013 03:05pm Have any canine or feline breeds shown a predisposition to pemphigus foliaceus? Also wondering if animals with tender pink skin seem prone? Reply to this comment Report abuse 10 Dr. Jennifer Coates 08/14/2013 04:36pm Akitas and Chows are at highest risk. I'm not aware of any breed predisposition in cats. Reply to this comment Report abuse 4 TheOldBroad Testing 08/14/2013 06:12pm Usually, if one of my critters goes to the doctor, they get a full blood panel. That has quickly found a few problems that hadn't been symptomatic yet.

Would it help at all to take skin scrapings, blood and urine on the first visit?

Also, since cats usually don't tolerate long-term use of steroids as well as it appears to be widely believed (think: steroid induced diabetes), what would be a long-term approach for cats with pemphigus foliaceus?

If left untreated, can there be other health effects? Reply to this comment Report abuse 9 Dr. Jennifer Coates 08/16/2013 10:03am If the owner is willing to foot the bill the "shotgun" diagnostic approach can be taken (or if the pet's condition is severe enough that waiting is not an option). Prednisolone used at a low dose no more frequently than every other day is safe and effective in many kitties, otherwise immunosuppressives like cyclosporine or chlorambucil can be used. Reply to this comment Report abuse 3 ASDMarlene My Experience 08/15/2013 11:45pm One of my dogs developed Pemphigus last year, well, we did not do biopsies to confirm that, but neither my regular vet nor the dermatologist have been able to come up with what else it could have been as it was only treated with prednisone. Also from my experience, there is no way I would have sat around for 2 to 3 weeks to run tests before starting an effective treatment. It was horrible enough despite my early intervention.
Since I was off work when this started, I know how this progressed hour by hour, truly scary. It started out with her having a small sore above the eye, which initially I thought she had gotten into a scuffle with another dog, though when I looked her over I didn't find the usual slime that goes along with dogs getting into a scuffle, I looked over the dogs she had been with and no sign of a scuffle. Since it was very small I left it alone. About 2 hours later I noticed a raised area on the bridge of her nose about 1 inch x 1 inch, I thought that confirmed a scuffle as I thought it was some bruising from a nip, couldn't find anything else in that area. A few hours later that swelling had doubled in height and the area was about 1.5 x 1.5 inches. So I gave her some Meloxicam before going to bed thinking an anti-inflammatory should help. In the morning the spot over the eye and on the nose had opened up and were now oozing bloody liquid and getting crusty. Plus she was getting more pustules in the corners of her eyes, so I am thinking maybe something bit her and she is having an allergic reaction so I gave her benadryl, when it didn't improve I gave her more benadryl a couple hours later. The sore on her nose started to look real oozy and crusty, I was wondering why she didn't seem to be itchy, she got a few more pustules around the lips and all of those pustules started to break open within hours of appearing. I could not get her to the vet because her sister just had TPLO surgery a few days ago and I had nobody to stay with her to make the trip to the vet (140 some miles roundtrip). So as a last resort I gave her prednisone and an antibiotic in the evening and expected that it would be a lot better in the morning. But it wasn't, it was about the same but I continued to notice a new pustule here and there around her muzzle, lips and eyes. By this time I knew this was something much worse than I had ever encountered. I called the vet and told them I would be there in 2 days (when my husband would be home to stay with the dog recovering from surgery). I continued the prednisone and antibiotic as at least it wasn't getting much worse since I started that, though the morning I took her to the vet I noticed another large pustule forming on her nose. I looked this up on the internet and concluded it must be pemphigus. When the vet looked at her and I told her what happened and what I have been giving, she seemed to hesitate to tell me what she thinks it is. But when I told her what I think from what I saw on the internet she agreed that it very much looked like it is pemphigus. We did some cultures and scrapings. I also told her that 6 weeks ago her thyroid level came back low and the same day we had drawn the labs she had received a parvo/distemper vaccine (she was 4 years old at the time) and that we had not treated her for hypothyrodism yet because we wanted to re-check it, but that at this point I would rather start her on meds instead of waiting to re-check. So she was started on soloxine that day. My vet recommended for me to continue the meds I had already started saying "you did exactly what I would have done" to see how she responds. I am happy to say that it did not get worse from that point on, not a single new lesion. I had to stop the antibiotics after less than a week because she started not eating and throwing up, so she only got the prednisone from then on. The smaller lesions healed up within a couple weeks, the large area on her nose took almost 2 months to be completely covered with new skin. She was on the original dose of pred for about 2 weeks and then we tapered it off slowly over the next 4 weeks. She has been in remission for over a year. My vet told me no more vaccine and I have a waiver for her rabies vaccine. I am hoping that being hypothyroid (not treated) and getting the parvo/distemper was the trigger and that treating the hypothyroidism and not giving vaccines will keep her in remission. Autoimmune disease is genetic and I found out later that her father also developed pemphigus and he was diagnosed via biopsy. However autoimmune disease is not passed on like other diseases, it is more likely, that it has to do with dogs inheriting the same genes in the Major Histocompatability Complex, which at this point testing breeding dogs for that is still in its infancy and even if testing is done there is not a guarantee that autoimmune disease will not happen. Much more research needs to be done in this area. Reply to this comment Report abuse 4
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