# pemphigus erythematosus



## Steve Estrada (Mar 6, 2011)

Easy for you to say! Never heard of it until yesterday when two pups I bred were diagnosed with it through a biopsy. This link is what I found most informativehttp://answers.google.com/answers/threadview?id=405349 
Some of the other reading might as well been in Chinese which I'm sure we all are about to learn. Does anyone have any experience with this, I'm about to take a nice bitch out of the gene pool over it. I'm curious to competency of diagnosis, alternatives etc. the dam in question shows nor has shown symptoms nor have other offspring from same litter nor other litters she has produced. Obviously I'm considering the stud dogs too. Without being rude I'm not interested in opinions unless they are founded. Thanks in advance


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## Joby Becker (Dec 13, 2009)

Steve Estrada said:


> Easy for you to say! Never heard of it until yesterday when two pups I bred were diagnosed with it through a biopsy. This link is what I found most informativehttp://answers.google.com/answers/threadview?id=405349
> Some of the other reading might as well been in Chinese which I'm sure we all are about to learn. Does anyone have any experience with this, I'm about to take a nice bitch out of the gene pool over it. I'm curious to competency of diagnosis, alternatives etc. the dam in question shows nor has shown symptoms nor have other offspring from same litter nor other litters she has produced. Obviously I'm considering the stud dogs too. Without being rude I'm not interested in opinions unless they are founded. Thanks in advance


Have never heard of it either, I did get a puppy, with Juvenile cellulitis, ended up having to be euthanized. I never heard of that either until then.

sorry to hear it man...

what is the hereditary aspect of this?


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## Steve Estrada (Mar 6, 2011)

It is genetic & I'm trying to chase it with little luck. I'd really know how to genetically link it. I'm not throwing baby out with bath water until I know more. It's found in GSD one site says usually males yet it's shown up in m&f both. I thought for sure someone here would know of it. Thanks Joby....


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## Maren Bell Jones (Jun 7, 2006)

Pemphigus is a pretty crappy and painful disease. It happens in humans as well as animal species. The second word after pemphigus describes what layer of the skin is affected by the immune system attack. It's diagnosed by a pathologist via biopsy, so that is the best way to diagnose it. It is treated and managed with long term use of a high dose of steroids or immunosuppressing drugs and often antibiotics to control secondary infections. This is both expensive and time consuming to treat, as well as there being many serious side effects possible with treatment. Euthanasia is not uncommon either from the disease itself or the side effects of the drugs used to treat the patient.

Here's a picture of a chow with pemphigus erythematosus (FYI: it's pretty painful to look at):

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1255596/figure/f1a-pg925/

Here's some more general information on the pemphigus complex:

http://www.veterinarypartner.com/Content.plx?P=A&S=0&C=0&A=2202

Doing some reading, I am not seeing anything to indicate that it is sex linked, but it is breed linked in German shepherds indicating a genetic predisposition. There are occasionally exposures to certain drugs that can cause an eruption, but two pups in the same litter (presumably not in the same house?) would probably rule this out. I would certainly not repeat a breeding between the two and would be exceptionally cautious breeding either the male or female again.


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## Steve Estrada (Mar 6, 2011)

Thanks Maren, you just threw the curve I was looking for because they are both in the same house and I do not see anything of it in their siblings or other breedings from Dam. My quandary is she is a great producer & awesome herself, how do I see if their is a genetic link as I've questioned the owners of three of her generations the best I'm able. I have a planned breeding that I can't conscientiously breed until I know more. Can I trace a genetic marker? Just trying to do the responsible thing. What's even more puzzling is no one on this forum or anyone I have talked with have ever heard of it in GSD....


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## Connie Sutherland (Mar 27, 2006)

Steve Estrada said:


> It is genetic & I'm trying to chase it with little luck. I'd really know how to genetically link it. I'm not throwing baby out with bath water until I know more. It's found in GSD one site says usually males yet it's shown up in m&f both. I thought for sure someone here would know of it. Thanks Joby....


_
"one site says usually males"
_
Where did you read this? I can't find anything saying that there is a sex link.



ETA

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1255596/

http://veterinarymedicine.dvm360.com/vetmed/article/articleDetail.jsp?id=653149

http://www.akitaclub.org/health/health/pemphigus1.html


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## Maren Bell Jones (Jun 7, 2006)

Steve Estrada said:


> Thanks Maren, you just threw the curve I was looking for because they are both in the same house and I do not see anything of it in their siblings or other breedings from Dam. My quandary is she is a great producer & awesome herself, how do I see if their is a genetic link as I've questioned the owners of three of her generations the best I'm able. I have a planned breeding that I can't conscientiously breed until I know more. Can I trace a genetic marker? Just trying to do the responsible thing. What's even more puzzling is no one on this forum or anyone I have talked with have ever heard of it in GSD....


I think that's very good you are trying to get to the bottom of this. I am not aware of a genetic test for it yet. I would have the owners consult with a board certified veterinary dermatologist to tease apart if there is some other issue going on. They can find one here:

https://www.acvd.org/locator/locator.asp


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## Steve Estrada (Mar 6, 2011)

Thanks again Maren I know of a place I have referred clients. Connie I can't tell you where I've read so much researching this, but I also read something counter to it also. I can't remember what I had for dinner last night so no surprise 
I'm looking for their health records to find if their is something prescribed that could have created this; obviously I think a lot of my female.....


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## Connie Sutherland (Mar 27, 2006)

Maren Bell Jones said:


> I think that's very good you are trying to get to the bottom of this. I am not aware of a genetic test for it yet. I would have the owners consult with a board certified veterinary dermatologist to tease apart if there is some other issue going on. They can find one here:
> 
> https://www.acvd.org/locator/locator.asp



Maren, it's driving me crazy that I know I have read of something (something rare) that mimics canine pemphigus erythematosus. I can't find it now that I want it. Any idea?


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## Maren Bell Jones (Jun 7, 2006)

Maybe of Promeris the flea/tick product? It was pulled off the market a while back because it was causing pemphigus lesions in a small number of dogs. There are other drugs out there that can cause rare reactions that manifest in the skin called drug eruptions, but this was a more recent case.


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## Connie Sutherland (Mar 27, 2006)

No, I think it's some kind of hard-to-see fungus .....


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## Connie Sutherland (Mar 27, 2006)

Found it (finally): acantholytic dermatophytosis

http://www.ncbi.nlm.nih.gov/pubmed/17610488
_
"Acantholytic dermatophytosis is a rarely reported condition of dogs that clinically and histopathologically mimics superficial pemphigus (erythematosus, foliaceus)."_


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## Steve Estrada (Mar 6, 2011)

All I can say is WOW! this will be forwarded to the lab & prescribing vet for further analysis with what I hope is a successful outcome. I have to say I started this thread with the hope that something would materialize & it seems to have. I also posted it for all our education& hope it in some way has or will help others. Thanks again all that have contributed I will update when available.


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## Richard Ramirez (Sep 6, 2011)

Good luck with the treatment Steve! Richard


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## eric squires (Oct 16, 2008)

Get hold of the people at Vetnostic laboratories. Vetnostic.com they are super and will help. They are willing to develop tests if there is evidence of this being a genetic issue. Call them and leave a message they will return it.


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## kenneth roth (Jul 29, 2010)

Did you find out what caused the pups to get this? how are the pups doing,mom?


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## Steve Estrada (Mar 6, 2011)

Pups are doing fine doesn't seem to be as big a deal as I have read but I have retired Mom for what; ethical reasons & some doubt. Really sucks sire is 2001 Bsgr Bastian v Kolkeltal x a Tom van't Leefdalhof dau. which is just frosting as this is a very serious female but so well balanced. Oh the joys of dogs & breeding, but she is still great, thanks everyone....


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## Peter Cavallaro (Dec 1, 2010)

good on you man for being up front about a possibly genetic disorder on a public forum, you pass my ethics test. most would just blame the new owner and try hose the whole thing down and keep breeding for a buck.

sorry this happened to you.


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## Maureen A Osborn (Feb 12, 2010)

Maren Bell Jones said:


> The second word after pemphigus describes what layer of the skin is affected by the immune system attack.
> 
> Actually, it is just refering to reddening of the skin, not which layer. Comes from the word erythema, meaning reddening of the skin.
> *adj. erythematous* - relating to or characterized by erythema
> ...


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## Maren Bell Jones (Jun 7, 2006)

No, ALL types of pemphigus show erythema as a very common sign. The second word (foliaceous, vulgaris, erythematosus) defines which layer of the desmosomes are attacked. I guess I could keep arguing with you, but I forgot you apparently went to a nursing school that also gives out concomitant DVMs. My mistake.


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## Maureen A Osborn (Feb 12, 2010)

Actually, there are 2 classifications in dogs, depending on which protein it affects: foliaceus is desmoglein 1 and vulgaris is desmoglein 3. Desmoglein 1 is found more superficial than Desmoglein 3. Desmogleins are the cement that hold the desmosomes together. Merck only classifies the 2, however, I have found where it says erythmatosus is a localized form of foliaceus and vegetans is a localized form of vulgaris.


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## Maureen A Osborn (Feb 12, 2010)

Actually, there are 2 classifications in dogs, depending on which protein it affects: foliaceus is desmoglein 1 and vulgaris is desmoglein 3. Desmoglein 1 is found more superficial than Desmoglein 3. Desmogleins are the cement that hold the desmosomes together. Merck only classifies the 2, however, I have found where it says erythmatosus is a localized form of foliaceus and vegetans is a localized form of vulgaris.

So you are kind right. But if you break down the words you get : *erythematous pemphigus* Etymology: Gk, _erythros,_ red, _pemphix,_ bubble . By definitiion,erythematosus has nothing to do with what layer it affects.


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## Steve Estrada (Mar 6, 2011)

Peter Cavallaro said:


> good on you man for being up front about a possibly genetic disorder on a public forum, you pass my ethics test. most would just blame the new owner and try hose the whole thing down and keep breeding for a buck.
> 
> sorry this happened to you.


Dog gone it Peter why didn't you let me know sooner I could've blamed the owner never thought of it. Oh well cat is out of the bag is that politically correct here 
Thanks though I can wish that everyone could find it in themselves but I can only be responsible for myself; that's hard enough most of the time :-({|=


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## Maureen A Osborn (Feb 12, 2010)

http://classes.kumc.edu/som/CellBiology/connections/intermediate/tut2.html










FYI Maren, you originally said it refers to what layer of skin, then changed it to desmosomes...not quite the same. Desmosomes is a general term and they are found in all layers of the skin. The differences are what the binding proteins are, let them be desmogleins and desmocollins.


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## Maureen A Osborn (Feb 12, 2010)

actually, have to correct myself, here is the definition of : *desmosome* (Greek: _desmos_, band, _soma_, body), also known as *macula adherens* (plural: *maculae adherentes*) (Latin for _adhering spot_), is a cell structure specialized for cell-to-cell adhesion. Does not just pertain to the skin, but also muscle.

Desmosomes are molecular complexes of cell adhesion proteins and linking proteins that attach the cell surface adhesion proteins to intracellular keratin cytoskeletal filaments.
The cell adhesion proteins of the desmosome, desmoglein and desmocollin, are members of the cadherin family of cell adhesion molecules. They are transmembrane proteins that bridge the space between adjacent epithelial cells by way of homophilic binding of their extracellular domains to other desmosomal cadherins on the adjacent cell. Both have five extracellular domains, and have calcium-binding motifs.
The extracellular domain of the desmosome is called the Extracellular Core Domain (ECD) or the Desmoglea, and is bisected by an electron-dense midline where the desmoglein and desmocollin proteins bind to each other. These proteins can bind in a W, S, or λ manner.
On the cytoplasmic side of the plasma membrane, there are two dense structures called the Outer Dense Plaque (ODP) and the Inner Dense Plaque (IDP). These are spanned by the Desmoplakin protein.[1] The Outer Dense Plaque is where the cytoplasmic domains of the cadherins attach to desmoplakin via plakoglobin and plakophillin. The Inner Dense Plaque is where desmoplakin attaches to the intermediate filaments of the cell.

If the connecting adjacent epithelial cells of the skin are not functioning correctly, layers of the skin can pull apart and allow abnormal movements of fluid within the skin, resulting in blisters and other tissue damage. Blistering diseases such as Pemphigus vulgaris and Pemphigus foliaceus are autoimmune diseases in which auto-antibodies target the proteins desmoglein 3 and desmoglein 1 respectively. The symptoms of the diseases are caused by the subsequent disruption to the desmosome-keratin filament complex leading to a breakdown in cell adhesion. Similar outbreaks occur with Hailey–Hailey disease, though the cause is not autoimmune, but, rather genetic. A haploinsufficiency of the ATP2C1 gene located on chromosome 3, which encodes the protein hSPCA1, causes malformation of the desmosomes


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## Steve Estrada (Mar 6, 2011)

Wow I wish someone would speak English here and I read that several times. To throw more fuel I spoke with another vet who thinks it may not be what I questioned so I will now delve further but keep this updated. Right when I was ready to throw the baby out with the bath water....


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## Maureen A Osborn (Feb 12, 2010)

Steve,you said it was diagnosed from a biopsy, but what signs/symptoms prompted the biopsy? Pemphigus(both types) can be diagnosed through a blood test looking for the antibody to the desmoglein 1 and desmoglein 3.


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