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Pemphigus Foliaceus
  

 


 

by Arnold Plotnick MS, DVM, ACVIM, ABVP

Pemphigus. It’s a horrible sounding word that most cat owners have probably never heard. Barnee Escott, however, was introduced to the word at her veterinarian’s office a few months ago, and she’d be happy if she – and her cat Oliver – never hear it again.

Oliver was a rescue cat, adopted by Escott from a local sanctuary. Everything about Oliver was pretty darn cute, except the thick grey scaly patches on both of his ears. At his first post-adoption veterinary visit, ringworm – a common skin fungus – was suspected as the cause of his ear condition, and a fungal culture confirmed the diagnosis. Treatment of the ringworm was successful, but the ear lesions never fully resolved. In fact, new scaly patches were cropping up - on his feet! The distribution pattern of Oliver’s skin lesions lit a light bulb over his vet’s head: perhaps we were dealing with an auto-immune skin disorder.

Auto-immune disorders are those in which an animal’s immune system mounts an attack against itself. The pemphigus group of diseases is a set of auto-immune skin diseases that cause blisters and erosions of the skin, as a result of the immune system mistakenly regarding the cells in the skin as being foreign, and attacking them. The pemphigus diseases are the most common auto-immune skin diseases recognized in cats and dogs. One particular disorder, pemphigus foliaceus (pronounced pem-fig-us fō-lee-ā-shus, and abbreviated PF in this article), is the more common of these feline skin disorders.

There is no age or breed predilection for PF in cats. One report showed the median age of onset to be 5 years, although it has been reported in cats as young as 1 and as old as 17. There is also no sex predilection – males and females are equally affected.

The head is the most commonly affected area. Cats with PF often show signs of the disorder on the ears, nose, chin, and around the eyes. The lesions tend to be bilaterally symmetrical, i.e. whatever is present on the one side of the head tends to also be present in mirror image on the other side, more or less. The footpads, nail beds, and area around the nipples are also commonly affected. Foot involvement is common, with multiple toes and multiple feet affected. In some cases, systemic signs of illness may be present, including fever, lethargy, weight loss, poor appetite, and enlarged lymph nodes. Cats with foot involvement may also show lameness.

The lesions caused by PF can resemble those of other diseases, including ringworm, bacterial skin infections, parasitic skin infections, inhalant allergies, and food allergies. Various tests, such as a skin scraping and fungal culture may be necessary to rule out parasitic and fungal infections. To obtain a definitive diagnosis of PF, skin biopsy specimens are required. The best specimens are those areas of skin that contain an intact pustule, however, these are hard to find, as pustules rupture easily. On occasion, a preliminary diagnosis may be made by the veterinarian via cytology. For example, if an intact pustule is present, it may be possible to puncture the pustule, collect the contents, and evaluate it on a microscope slide. The presence of a particular type of cell, called acanthocytes, is highly suggestive of the disorder. This preliminary diagnosis should be confirmed, however, by biopsy. This test also helps rule out other pustular skin diseases, like pyoderma (bacterial infection of the skin). Submitting multiple biopsies increases the chances of obtaining diagnostic sample. It is important that the biopsy specimen be evaluated by a pathologist with particular expertise in skin disorders. Oliver’s vet performed biopsies on the affected areas of Oliver’s skin, and as you’ve probably figured out by now, pemphigus foliaceus was the final diagnosis.

“My initial reaction was shock”, said Escott. “I have had cats for 40 years and never even heard of pemphigus!” But she was undaunted. “I decided I wanted to give him the best quality of life that I could, for as long as possible”, she said.

Treatment of PF usually involves administration of oral corticosteroids. Initially, high doses are used to get the disorder under control, and then the dose is gradually tapered, with the goal being the lowest possible alternate-day dosage that controls the clinical signs. Oliver was treated with prednisone, a commonly prescribed corticosteroid. Potential side effects of corticosteroids include excessive thirst and urination, increased appetite, weight gain, and increased risk of infection. Prolonged use can predispose some cats to developing diabetes. Fortunately, cats are fairly resistant to the adverse effects of steroids, especially when compared with dogs.

If steroids alone fail to produce remission, or if unacceptable side effects occur, alternative or concurrent medications can be administered. Chlorambucil, a chemotherapy drug, is a good second drug to try if steroids alone produce inadequate results. In fact, some veterinarians initially treat with both drugs, and then taper the drugs so that the cat is maintained on alternating day therapy, with steroids given one day, and chlorambucil given the other day. In some instances, the steroids can be completely discontinued and the disease kept under control on chlorambucil alone. Another drug that can be tried are gold salts. They are available orally, or as an injection. The injectible form is thought to be more effective, although the drug is expensive and can be difficult to obtain. Injections are usually given weekly until remission occurs (usually 6 to 12 weeks), and then decreased to every two weeks, and then every one to two months.

Oliver responded well to high doses of corticosteroids, but when tapered to every other day therapy, his skin flared up again. His steroid dosage was increased; the PF went back into remission, and chlorambucil was administered concurrently. After two months, Escott was able to reduce the prednisone and chlorambucil. It’s been three years since Oliver’s diagnosis, and he continues to do well, despite the subsequent development of lymphocytic plasmacytic gingivitis/stomatitis, a painful mouth condition (see my article “Oral Agony” in the July 2006 issue of Catnip for more details about this disorder). When his mouth flares up, it becomes difficult to medicate him orally, and some of his medications are given by injection instead. This protocol seems to be working quite well, and Oliver shows no signs of slowing down.


        

Updated 7/2/07