What is Pemphigus? Simply put, it's an Autoimmune Disease

PEMPHIGUS is a skin disease of dogs and cats that typically begins in early middle age. It causes pustules and crusting on the skin surface. The skin under these crusts is raw and may be painful. The disease often begins on the face, bridge of nose and ears, but often spreads to affect the skin on other parts of the body.


Pemphigus is the general designation for a group of autoimmune skin diseases involving ulceration and crusting of the skin, as well as the formation of fluid-filled sacs and cysts (vesicles), and pus filled lesions (pustules).   Some types of pemphigus can also affect the skin tissue of the gums.

 

An autoimmune disease is characterized by the presence of autoantibodies that are produced by the system, but which act against the body’s healthy cells and tissues — just as white blood cells act against infection. In effect, the body is attacking itself. The severity of the disease depends on how deeply the autoantibody deposits into the skin layers. The hallmark sign of pemphigus is a condition called ACANTHOLYSIS, where the skin cells separate and break down because of tissue-bound antibody deposits in the space between cells. 

Pemphigus is more often, at least from my experience with Jake and others who have gone through the process, misdiagnosed as a bacterial or fungal infection.  This is especially true if your dog has a history of skin issues (allergies, bacterial, fungal, yeast infections, etc.)  This post will focus on what Pemphigus is, what the symptoms are and what to look for, how to manage and treat and what to expect.  You'll also find photos of Jake's journey with Pemphigus so that you can see what he looked like.  

Please comment below with any questions you have or to let us know if this information has helped you.  Pemphigus is very near and dear to our hearts.

 So Why Is Pemphigus So Misdiagnosed?

As you can see from these two photos, there is nothing specific about the disease that would lead anyone initially to think that this is anything more than a skin infection, either bacterial or fungal.  Around April/May 2013, Jake started to present with signs of what looked like a typical skin issue for him.  He was prescribed some antibiotics and a short round of steroids to get it under control.  This protocol worked, until it didn't.  About 21 days later, after showing some signs of improvement, it only grew angrier.  And the typical treatment protocol wasn't working.  Antibiotics will NOT do anything to deter Pemphigus.  Because it is an autoimmune disease, the only protocol that will work is one that utilizes immunosupressant therapy.  


But Wait! There are Some Tell Tale Sings in These Photos.

The bottom photo is the one to pay close attention to.  Why?  Because Pemphigus does in fact have a tell tale sign.  It starts to rear its ugly head along the bridge of the nose, onto the forehead, top of head and the outline of the ear.  A trained professional SHOULD have caught this.  You'll find out the EXACT reason they didn't as you read on.

does my dog have pemphigus, pemphigus foliaceous
Pemphigus Signs, Pemphigus Symptoms, Dog with Pemphigus


There Are Four Types of Pemphigus:

Pemphigus Foliaceus, Pemphigus Erythematosus, Pemphigus Vulgaris, and Pemphigus Vegetans.  This post will focus solely on Pemphigus Foliaceous as this was my experience with the Foo.  

If you're here reading this post, you've most likely received the Pemphigus diagnosis or suspect your dog has it.  Do not dispair.  This disease is treatable and manageable, and becoming more mainstream in the last few years as more vets are gaining experience with it and available treatment options.

Here are 4 photos showing the progression of Pemphigus in Jake from April 2013 through October 2013, the entire time he was misdiagnosed.  These photos are intended for illustrative purposes and not intended to diagnose your dog.  The only way to get a proper diagnosis is through a skin biospy.  More on that below.  

dog with pemphigus, bacterial infection in dog

 

Symptoms | Pemphigus Foliaceous

    • Scales, crust, pustules, shallow ulcers, redness, and itching of the skin
    • Footpad overgrowth and cracking
    • Fluid-filled sacs/cysts in the skin (or vesicles)
    • The head, ears, and footpads are the most commonly affected; this often becomes generalized over the body
    • Gums and lips may be affected
    • Swollen lymph nodes, generalized swelling, depression, fever, and lameness (if footpads are involved); however, patients often are in good health otherwise
    • Variable pain and itchy skin
    • Secondary bacterial infection is possible because of cracked or ulcerated skin

Diagnosis

Your veterinarian will perform a thorough physical exam on your dog, including a blood chemical profile, a complete blood count, a urinalysis and an electrolyte panel. Patients with pemphigus will often have normal bloodwork results. You will need to give a thorough history of your dog’s health and onset of symptoms. Possible incidents that might have precipitated this condition should also be reported to your veterinarian (e.g., exposure to sun, excessive use of steroids).

{a skin biopsy is crucial}

A skin biopsy (not a skin scraping) will be taken from an infected area on your dog; and pustule and crust aspirates (fluid) should be wiped onto a slide to diagnose pemphigus. A positive diagnosis is achieved when acantholytic cells (i.e., separated cells) and neutrophils (white blood cells) are found. A bacterial culture (swabbing or scraping) of the skin may be used for identification and treatment of any secondary bacterial infections, and antibiotics will be prescribed in the event that there is a secondary infection present.

Your dog will need to be off steroids for at least 4 weeks prior to the skin biopsy or you could get a false negative reading from the biopsy; meaning they tell you your dog doesn’t have Pemphigus, even though they really do.

This was what happened to Jake.  Twice.  And what really made me MAD was that they were ALL AWARE that he was on Prednisone and should have caught this. But in any case, always alert your vet to any and all medications that your dog is taking prior to any test. Sometimes they need a reminder, and if it’s a new vet you’re seeing, they aren’t too familiar with your dog’s history.

Good News!  It’s Treatable.

Your dog may need to be hospitalized for supportive care if it is severely affected by the condition. This is what happened to Jake.  From being misdiagnosed for 6 months, the condition had gone septic (into his bloodstream) and created an entire host of other problems for him.  It is imperative to get this treated quickly.  Steroid therapy may be prescribed briefly to bring the condition under control. If corticosteroid and azathioprine therapy is prescribed, your dog will be switched to a low-fat diet, since these medications can dispose animals to pancreatitis. Your veterinarian will treat your dog with the drugs that are specifically suited to the form of pemphigus it has.

 pemphigus stages in dogs

Living and Management

Your veterinarian will schedule follow-up appointments to see your dog every one to three weeks. Standard blood-work will be performed at each visit to check for progress. Once your dog’s condition has gone into remission, your dog may be seen once every one to three months. The sun can worsen this condition, so it is important to protect your dog from excessive exposure to the sun.  Jake never went outside without wearing light clothing and sunscreen and enclosed in his stroller, a.k.a. Rolling Man Cave.  His outside time was severely limited to potty breaks once he was diagnosed.

His diet was fairly simple, as a low-fat diet is required.  He was fed wild-caught Salmon, mixed veggies, sauteed in coconut oil.  He ate twice a day and had dehydrated snacks for treats/cookies.  Jake was a total STINKER about taking his medication, which was crucial.  He couldn’t miss a dose.  The only way he would take his medication was in a jelly munchkin from Dunkin Donuts.  I tried everything else, he’d spit it out.  So we became regulars at the drive through, and twice a day, Jake would take his meds, inserted into a jelly munchkin.  I spoke with me vet about this and he advised me that one munchkin a day would not hurt him or affect his system.  While I don’t advise this for a dog who must be on a strict diet, you must consult your vet.  I would never push a pill down his throat as I think it is extremely inhumane, and this worked for us.

Side Note

Jake was put on Prednisone (steroids), Atopica (a generic and more affordable brand of the same medication is Cyclosporine), Pentoxyfilin (something that is rarely prescribed as it was fairly new and cutting edge; used for treating skin conditions), and Azathioprine (an immunosuppresant).  He responded exceptionally well to all of the medications.   It is imperative that if administering Atopica (or the generic version - Cyclosporine) that it be given on an empty stomach. If given with food, or after a meal, your dog will most likely experience an upset stomach.  This happened to Jake.

The medications prescribed to Jake are pretty standard in the vet arsenal for treating Pemphigus; however, your vet may add or delete certain medications.  This is the protocol that worked for Jake.  If your vet has prescribed something different and it's working, terrific!  If not, suggest they look into this treatment to see if it's a viable option for your dog.

In addition to the medications required to suppress his immune system, I added natural supplements to Jake's daily intake -  Milk Thistle and Bromelein to flush the toxins (from the medications) out of his system, mainly his liver where they tend to collect.  You can ask your vet for proper dosing amounts as it is specific to weight.  Additionally, I added Fish Oil and Coconut Oil to his food.  These oils helped to create a stronger and healthier coat as his hair started to grow back in.  He also used (what would become My Dog Foo's) Soothie Woothie™ for breakouts to help calm and ease his flare ups, and this provided valuable antibacterial protection to any open sores.  It was super important that any open wounds and/or lesions were coated in Soothie Woothie™ to prevent infection from creeping into his suppressed immune system.  It also provided some sunscreen protection when he went outside for brief periods, during potty breaks.  

I also bathed him in what would become My Dog Foo's Healing Shampooch Bar™ to soothe and heal his skin and to help stimulate his hair follicles to grow.

So If It’s Treatable, Why Do Dogs Dies From It?

Sadly, most dogs will die from Pemphigus for two reasons:  misdiagnosis and cost.

Let’s Look at Misdiagnosis First.

Often, due to lack of experience with this particular autoimmune disease, many vets will diagnose initial outbreaks as bacterial or fungal infections, sending them on their way with medications and special shampoos.  This is exactly what happened to the Foo.  While this will work initially, in time, these medications and baths will stop working because they aren’t targeting the root of the problem- the immune system.  And the chemicals in these medicated shampoos are so harsh that they only aggravate the skin and make it worse.

Now Onto Cost.

Initially, Jake’s medications when he was first released from ICU ran about $700-800 a month.  He also needed cold laser therapy and water therapy for secondary issues, which put his care around $900-1000 a month for the first 2 – 3 months. Because the cost of treatment can be high, many dog owners will opt to euthanize their dog.  So, the dog isn’t really dying from the disease, but rather by the hands of an owner who is unwilling to find a solution to provide care.  

Yes, it can be expensive.  And if cost is something that concerns you, I would suggest reaching out to local rescue groups who may be able to offer assistance.  Start an online fundraiser, ask for donations.  Do whatever you can for your dog.  Pemphigus can be extremely painful.  Dogs by nature rarely let you know they are in pain.  So it is important that you start to address the condition promptly.

Here are some photos of jake as he went through his recovery and into remission. He still had some light scabbing and crusting at times, which I lovingly called his "cornflakes".  But you will see that with proper care and treatment, this can be treated effectively and, with proper care, the disease will go into remission.

Pemphigus Signs, Pemphigus Symptoms, Dog with Pemphigus

If you suspect your dog is suffering from Pemphigus, I urge you to discuss a skin biopsy with your veterinarian.  A scraping will not detect this disease.  And, like what almost happened with Jake, it can be fatal if not properly diagnosed.

I am always happy to answer questions if you are dealing with or suspect your dog may have it.  While I am not a vet, and do not pretend to be one, I am happy to share our story if it will help others and share our experiences so that we can educate as many dog owners as possible about Pemphigus.

If you have experience with caring for a dog who has had Pemphigus or might think that your dog has it based on this information and the photos, please comment below.  My Dog Foo hopes that together we can raise awareness about Pemphigus and get more dogs properly diagnosed.

Additional Reading:

Pemphigus in Dogs

Canine Pemphigus – Otis’s Journey

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Information on the general disease was provided by PetMD.  Please also keep in mind that this was our experience with the disease.

 

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