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Autoimmune disease (Phemphigus) or....? Help!

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  • Autoimmune disease (Phemphigus) or....? Help!

    About 2 weeks ago, on December 5th, my horse broke out in severe hives. The right side of his body was covered in small hard hives and the left side of his body, mainly his stomach and side, were covered in, essentially, a giant inflamed hive. This is a 14 year old Thoroughbred gelding, schooling GP dressage and I've had him for 11 years. He has never been a skin sensitive horse, he has never broken out in hives before, and he has never, to my knowledge, had any sort of allergic reaction since I've had him all these years. He was extremely uncomfortable and we immediately gave him 5cc of Dexamethasone IV and hand walked him to try and get the inflammation down. The next day, the 6th, the hives were better but still present and so we dosed him again with 5cc of the Dex done IV.

    Friday, December 7th, the hives were down and he was no longer acting bothered or uncomfortable so we discontinued the use of Dex and just hand walked and lunged in order to try and increase the circulation. At this point, where the hives had previously been the skin was crusting from serum but he did not seem bothered by it at all, or in pain. December 8th he was acting fine and the hives had not reoccurred and so again, we did not give him any Dex and I rode him for a short 20 minutes and he felt absolutely normal. Sunday, the 9th, he had the day off.

    On Monday morning, Dec 10th, the hives had still not made a reappearance, however, where he had been most swollen the hair was beginning to fall out and he was pretty crusty, and his right hind leg was suddenly starting to swell and was extremely painful for him. He did not even want it to be touched and he would not trot out on the lunge line for more than three strides, which is very unusual for him since he is a rather forward horse with a high pain tolerance. He also had fairly severe edema on his stomach. The vet came to the barn and gave him a 4 day injection of antibiotics, pulled blood, gave us Naquadex to give him (5cc twice a day until edema and swelling in leg gone), and pulled skin biopsies on the assumption that he might have the autoimmune disease, Phemphigus Foliaceus.

    After sweating the leg with a Furazone/DMSO mix, the leg returned to normal and the hives had still not reappeared. On Dec 14th, after lowering the Naquadex to 5cc once a day, he rehived and the leg began to swell slightly, although he was not lame or sensitive to touching or pressure on it. We increased the Naquadex back to 5cc twice a day.

    His bloodwork came back as normal, and throughout this entire ordeal he never had a fever and remained fairly constant at 99.1 to 99.4. After two days of the Naquadex his hives went down and have not since reoccured, although he continues to lose hair from the inflammation and has one of two small hard hives with slight serum weeping.

    On Monday, Dec. 17 his skin biopsies came back as inconclusive on whether or not he had Phemphigus. The vet informed us we could have the biopsies resent to a specialist for confirmation or continue low dose Dex and see where that puts us. We decided to continue with 2.5 cc of Dex. He had 2.5 cc Dex on Dec. 17 and 18. On Wednesday the 19th we discontined the Dex and he has not been given any these past few days. Yesterday, Dec. 21st, however, he has now developed small hard lumps on his right hind fetlock, with a few occurring by the pastern and slightly up the cannon bone. None of his other legs have been affected with any swelling this whole time and none of his other legs have developed these bumps. We decided to bute and sweat the leg with Furazone and DMSO and continue to ride him since he was not sensitive to the bumps or lame.

    Today, the sweat wrap reduced any remaining residual swelling in that leg, but did nothing to the bumps. The bumps, now, are actually peeling off when pulled and leaving raw skin underneath. Again, my horse does not seem to be sore or all that perturbed about the bumps and what is going on with that leg. We sprayed with an anti-bacterial and anti-fungal agent and wrapped him and kept him inside this morning, although he was sound and energetic while riding this morning. I'm not sure if I should give him some more steroids, though I don't want to if I don't have to.

    We tried to call the vet yesterday morning and say that we do think we should send the biopsies out to a specialist, but they are being rather unhelpful and refuse to do it until after the holidays.

    Basically, while I'm waiting for any help from the vet and I've put in a consult with a better vet hospital since I need more help than I'm getting from my current vet, does anyone have any idea what that could possibly be? Have you seen anything like it before?

    If it sounds like something else, what on earth could it be? And while it very well might be Phemphigus, had anyone experienced this and knows what are the best protocols, supplements, and steroids to use?

  • #2
    I think you need fresh full thickness (punch) biopsies of the newest lesions and send out to a veterinary dermatologist/pathologist. Yes they exist! Contact your local vet school for info on who to send them to.

    You need a diagnosis; that is paramount for the proper treatment protocol. You don't want to take the risk of long term steroids unless it's absolutely necessary.

    Comment


    • #3
      Oh, and would love to see pics if you feel like taking some ....

      Comment


      • #4
        Not a freaking clue. I have never even heard of this and will be off to google shortly. But I wanted to send some jingles in hopes you'll get to the root of this and find a diagnosis and a treatment.

        My goodness.

        I think that in light of the "vacuum" of veterinary counsel you're getting right now, I'd be inclined to load up and go to the nearest U.
        A good horseman doesn't have to tell anyone...the horse already knows.

        Might be a reason, never an excuse...

        Comment

        • Original Poster

          #5
          The skin biopsies that were taken were punch biopsies, with a total of 4 of them. The inconclusive result could either be because he doesn't have it, or since he was given Dex three days prior to the biopsies it could have messed up the results. They could take new biopsies, however, his body has not rehived so I'm not sure if that would work...they would have to try and biopsy off the right hind leg around his fetlock, perhaps.

          Here are some pictures of the initial hiving, hairloss, and the feeling skin on the leg that has currently developed.

          One

          Two

          Three

          Four

          Five

          Comment


          • #6
            Originally posted by Rumours View Post

            Basically, while I'm waiting for any help from the vet and I've put in a consult with a better vet hospital since I need more help than I'm getting from my current vet, does anyone have any idea what that could possibly be? Have you seen anything like it before?
            Sad to say yes, I have dealt with the symptoms on a 12 yr old TB mare.
            It was numerous biopsies later that we finally had a answer and it was not what we or the vets expected at all, cancer of the lympnodes.
            This mare had many bouts of strange symptoms for several years and for the most part, the vets at Tufts kept thinking it was a dermatology issue, finally after what was about 3/4 biopsies later they did a deep lymp punch biopsy and by the time we got the answer the mare only lived for 3 more months. She was always the picture of perfect health as far as weight and bloom on coat but would have bouts of being very quiet than a day or so later would bounce back, plus the skin condition issues.

            Hope this is not your horse and you will get a happy answer and get it cleared up!

            Comment


            • #7
              I have not heard of this before, but did they do any cultures or slides from the "goo" from the hives? That may give some information too. Do you have a vet school nearby?

              Comment

              • Original Poster

                #8
                And the lympnodes were not swollen and the bloodwork was normal? If he had cancer, wouldn't the bloodwork show elevated counts?

                When we were using steroids (Dex) he seemed almost distant and not his usual boisterous self, but off the dex he is back to being more outgoing and obnoxious.

                Comment


                • #9
                  You can have cancer and not have enlarged lymph nodes, but I'm not sure that would be my first concern....again, not a vet nor do I play one on TV.

                  I just hope you can get to a doc who will work with you more closely. I do hope you have a good university nearby.

                  Please keep us posted. This is very interesting. (never good to be an interesting case though!)
                  A good horseman doesn't have to tell anyone...the horse already knows.

                  Might be a reason, never an excuse...

                  Comment


                  • #10
                    For those that do not know what this is and want to learn ... here's an article from the AAEP website:


                    PEMPHIGUS FOLIACEUS

                    By Ann Rashmir-Raven, DVM, MS, Dipl. ACVS

                    Skin Problems - Jul 25th, 2012

                    Horses commonly develop different types of dermatitis (simply, skin inflammation), and with early and appropriate diagnosis and treatment, the conditions often respond to therapy. But what happens when the scaling, crusting, or blisters your horse develops are more than just the average dermatitis?

                    Pemphigus foliaceus (PF) is an uncommon but potentially life threatening cause of skin disease in horses. It is an autoimmune disorder in which the affected horse makes antibodies against his own skin. Normally, antibodies help fight infection and keep the horse healthy. But with PF, the horse’s body creates antibodies against its own tissues, and they destroy the bonds that hold the horse’s skin cells together. When these bonds break, the skin cells separate from one another, leaving a space that fill with fluid and forms a blister or pustule. These blisters and pustules arise close to the horse’s skin surface and are very fragile; therefore, they rupture quickly. This leads to the common signs affected horses’ exhibit: oozing, crusting, scaling, and erosions.

                    Some horses’ signs begin with acute, recurrent, or chronic hives, and a number of affected horses lose significant amounts of hair. Lesions commonly begin on the face, legs, or abdomen and spread quickly over the next one to three months. Some horses develop lesions localized to the face or coronary band for prolonged periods. Many horses with PF have considerable lower leg and lower abdomen swelling, with or without painful, hypersensitive, or itchy skin. Some horses become so painful they are reluctant to move. A large number of PF horses also exhibit fever, depression, lethargy, anorexia, and weight loss.

                    The exact cause of pemphigus foliaceus remains unclear, although some experts believe black flies and sensitivity to Culicoides gnats can be inciting factors. In some horses, PF seems to worsen or recur during warm weather, suggesting that seasonal allergens such as pollens and ultraviolet light might also play a role in some cases. Veterinarians believe drugs such as antibiotics, dewormers, vaccines, and some supplements have also triggered a number of PF cases. Although genetic factors have not been implicated in the horse, the condition in other species does appear to have a genetic predisposition. Researchers report no predilection based on breed, age, or sex.

                    Some practitioners confuse the early stages of PF with allergic reactions such as drug or insect allergies, infections such as ringworm or rain rot, cancers such as lymphoma, and keratinization defects such as cannon keratosis (keratin—a protein that forms hair, skin, and horny tissue—growth on the horse’s legs). Late stages of disease can also be confusing, and veterinarians should make every effort (using special stains, fungal cultures, etc.) to rule out other diseases such as severe ringworm. Even the most seasoned horse owner or veterinarian might not recognize PF immediately, leading to a delay in treatment and resulting in a poor response even when the appropriate treatment is finally given.

                    Veterinarians diagnose PF based on the horse’s history, physical examination, direct smears from blister fluids, and skin biopsy results. A trained dermatopathologist should evaluate the skin biopsy and might request special staining or other tissue treatments to look for the antibody that attacks the horse’s cellular bonds.

                    Treatment consists of long-term glucocorticoid administration (prednisolone or dexamethasone), omega fatty acid and vitamin E supplementation, sunlight restriction, and addressing any underlying causative factors such as fly bite allergies, drug administration, and diet. Horses that do not respond to these approaches might require treatment with injectable gold salts, azathioprine, and/or oral pentoxifylline. Bathing the horse in cool water might make him more comfortable. Rarely, young horses with PF might recover on their own, but most horses will require long-term if not lifelong treatment. Younger horses also tend to have less severe disease than older horses, respond better to treatment, and might remain in remission even after treatment is withdrawn. Unfortunately, about 50% of horses that initially respond to treatment relapse—some immediately, others after several years. In general, horses become progressively less responsive to treatment with each relapse. Horses are frequently euthanized when treatments fail, if they develop laminitis secondary to treatment, or if treatment costs become prohibitive.

                    Comment


                    • #11
                      You might do a search as I know there have been two or three threads on PF.

                      Nine years ago, I had a pregnant mare come down with PF. It took us almost two mos. to get her diagnosed. Prognosis was poor, but since she was carrying Cyriz's first foal, we decided to give her a chance. Best case we were told was that we got to delivery and had an orphan to deal with.

                      She was on massive amounts of steroids, mainly prednisolone. This should have caused her to abort, but she held on....and has continued to hang on! She has had four foals and is carrying her fifth.

                      Here is a link to her and the daughter she was carrying:
                      http://www.debracysporthorses.com/De...ies_Story.html

                      One of the problems we had is that PF is very rare in horses, more common in certain breeds of dogs, so many vets never see a case in their entire career. She was diagnosed by Texas A&M. And I agree with the poster who said you need punch biopsies.

                      Good luck!
                      www.debracysporthorses.com
                      Home of Sea Accounts xx
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                      Comment

                      • Original Poster

                        #12
                        The biopsies done were, in fact, punch biopsies. There were four in total, one on his left front shoulder, one on his right lower stomach (a place where much of the lower edema was occurring), on on his right hind, and then one on the left side of his stomach about 4 inches down from his unclipped saddle patch.

                        These were the biopsies that were sent out to the lab, though not the specialist, and they came back as inconclusive for Phemphigus.

                        Comment


                        • #13
                          I would try and get the samples sent to the derm pathologist if possible.

                          You can also take him off all meds for a week or so and re-biopsy if you think the drugs interfered with the results.

                          Response to immunosuppressive treatment is your other option, but personally I wouldn't want to do that without a diagnosis. It isn't without it's own set of risks.

                          Have you tried a long term course of antibiotics, like 3-4 weeks of SMZ's? I see in your OP that he had one injection (probably Naxcel) but I didn't see a reference to oral antibiotics.

                          Comment

                          • Original Poster

                            #14
                            He has now been off of steriods (Dex) for a week, the last time he was given any was December 19th. So far, his body looks the same and he has not rehived at all. He still has the remaining hair loss but he is in good spirits and back to his normal behavioral self.

                            However, the bumps on his right hind are still present and now there are a few on his left hind. They are not open, or weeping or "sores," they are just bumps that when plucked at will pull away from the skin and bring the chunk of hair with them. My vet is still on vacation and won't be back till tomorrow, so I'm a bit at a loss of what to do. I'm been spraying with an anti-bacterial and anti-fungal spray treatment, and wrapping but so far there hasn't been any effect. He's not sore on the legs at all, he doesn't mind them being touched, and he's extremely sound.

                            I can't tell if this is just another sign of the Phemphigus, or if it's some strange fungal infection. I think I'm going to try treating with topical steroid cream today until I can hear back from the vet tomorrow. Any ideas?

                            Here are a few pictures of the leg bumps and of his hair loss, and one picture of us riding to show that he's happy and relaxed to work.

                            Leg

                            Leg

                            Right Flank

                            Right Stomach

                            Riding

                            Comment


                            • #15
                              I wouldn't do anything without your vet's approval. You'll need a pretty strong topical steroid if it's going to work; something like Panalog cream or Genesis spray. You may be able to pick up a small amount of Panalog from a small animal vet until your equine vet gets back. You might be able to buy Genesis over the counter.

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