In pemphigus, the autoimmune system is directed against the skin itself.  Affected dogs have what looks like a rash at onset that develops into blisters and then pustules.  Finally, the dog is left with crusty sores on the non-furred areas of skin--the pads, nose, lips, and eye rims.  Occasionally, all the skin is involved, but this is less common.  Because the produce pigment-production cells in the skin may be affected also, color may disappear from involved areas. 
   An affected dog may show signs that he doesn't feel well; he may seem tired and depressed.  He may also be stiff from swollen joints.  The disease shows no sex preference and is usually seen in mature dogs (over two).

Diagnosis
Diagnosis is made by skin biopsy.  Thyroid panels should be run also because pemphigus is affected dogs often also have thyroid disease and need supplementation.

Treatment
Affected dogs are usually maintained on steroids with the dosage being adjusted with the severity of symptoms.  If steroids alone are not effective, then other immunosuppressors can be used. Treated dogs can often live a fairly normal life, but the disease cannot be cured.  Affected dogs should certainly not be bred.

   This represents a preliminary attempt to define two diseases that assumedly have a genetic basis in the Akita Breed.  Although relatively uncommon, both are devastating because they affect young, adult Akitas: dogs that may already have been on the show circuit or perhaps, may even have been used for breeding.  These diseases are also important to define as neither has a safe, effective therapy.
   The first disease is a condition we call panepidermal pustular pemphigus (also known as pemphigus erythematosus). This is believed to be an autoimmune disease that generally starts on the bridge of the nose with crusts (scabs) and loss of pigmentation. As the lesions progress, the skin of the ears, around the eyes and, in severe cases, the entire body may be affected.
   The second disease, sebaceous adenitis is a condition whose cause continues to elude us.  The disease presents as scales (dandruff) that typically start on the face and ear tips but may involve large areas of the body, especially the back.  The most characteristic lesion is the presence of scales that tightly adhere at the base of hair shafts.  A disease with great variability in its presentation, cases can range from mild scaling and hair
loss to a condition in which there is almost complete hair loss and prominent
scales.
   We want to study these two diseases, but before that is possible, we need to have a bank of serum, whole blood and skin biopsies from affected and non-affected dogs.  The serum will be utilized to examine for the presence of
circulating antibodies to the epidermis (pemphigus) or sebaceous glands
(sebaceous adenitis).  The whole blood will be used to isolate DNA that will
be used to ultimately identify the gene(s) that are associated with these
disorders.  The skin biopsies will be used to confirm the diagnosis, define
the presence of antibodies deposited on the skin and to serve as an additional source of DNA.
   We have thought a lot about how to get the materials we need to begin this
study and have decided the best way is to work with Akita Breeders and Akita Owners.  We are doing this because the success of this study depends on getting samples before any steroid therapy has been instituted.  By educating the Akita Owner, we believe that they can inform their veterinarian about the study and by working with their veterinarian, can assure that samples are taken correctly and before steroids have been administered.

Samples should be sent to:

Akita Tissue Bank
Dermatopathology Specialty Service
215 VMA
College of Veterinary Medicine
Texas A & M University
College Station, Texas 77843-1147

Instructions and the answers to some faqs are below.  For further information or other inquiries, please email us: Robert W. Dunstan, DVM
or  Dr. Kelly M. Credille, DVM
 

• When should an Akita have skin biopsies and blood?
  • We would like you to consider submitting samples to our tissue bank if: 
    • You find that your dog is starting to develop facial crusts, often in a symmetrical pattern (pemphigus) or scales on the ear tips and trunk that are often adhered to hair shafts (sebaceous adenitis), and
    • your dog has not had any steroid therapy for over three months.
  • We recommend that the decision to submit samples be made only after consultation with your veterinarian, who can help you decide if either of these two diseases should be considered as the cause of your dog's skin lesion.
     
• How are tissue to be submitted?
  There are three different samples that should be collected:
  • Frozen serum--(1.5 ml minimum).  Whole blood should be collected in a 5 ml orange top Vacutainer tube, spun in a centrifuge, the serum removed and frozen. 
  • Whole blood in EDTA--(2.5 ml minimum).  Whole blood should be collected in a lavender top Vacutainer tube and kept cold but not frozen.
    • Formalin-fixed biopsies to confirm the diagnosis and provide additional tissues for immunohistochemistry and DNA isolation.

     There is a possibility that your veterinarian may not believe that a
  biopsy is needed initially and that therapy should be attempted before any surgery.  This should not pose a problem for our tissue bank because if pemphigus or sebaceous adenitis is present, a biopsy will eventually be needed to confirm the diagnosis.  Thus, there is no need to biopsy initially; however, if the lesions progress to where a biopsy is necessary, we would like to be able to evaluate the sample microscopically to confirm the diagnosis.
     Note that if it turns out that the dog does not have pemphigus or sebaceous adenitis, the samples are still of value because we can use them as controls.

• How do you send the samples?
  • The serum should be sent frozen in a Styrofoam container.  The best way to send frozen material is by packing it in dry ice (if available). If not, the samples should be wrapped in newspaper and packed with frozen cold pack.  Samples should be sent overnight delivery.
  • The EDTA blood should be sent either frozen or simply cold. It should not be sent at room temperature.
  • The skin biopsies should be sent in 10% neutral buffered formalin.  If you are sending the biopsied material during cold weather and the container might freeze, we recommend mixing 1 part alcohol (ethanol,  methanol or isopropyl) in 9 parts formalin.  If sending the formalin-fixed tissue with the blood, you should put the blood in a separate, sealed, plastic bag to avoid the hemolytic affect of formalin.
  • Samples should be sent with names and addresses of veterinarian and owner, dog's age, sex and a pertinent medical history including a description of the distribution and appearance of the lesions.
• What would this cost?

         We will perform the organization of samples and the isolation of
DNA from the EDTA tube at no charge. We will charge our usual rated for
interpreting the biopsy sample ($35.00)

From Linda Wroth, chair of the Health and Genetics Committee:
1997  (Note: In 1998, Dr. Dunstan began researching at Texas A & M University, College of Veterinary Medicine)

   In Spring/Summer Newsletter of The Genodermatosis Research Foundation (GRF) has an article entitled "Pemphigus - The Other Congenital Skin Disease of Akitas" by Dr. Robert Dunstan, DVM, MS, DACVP (who's head of GRF's Research Committee and a veterinary pathologist at Michigan State University). The article is short but has a plea for information on this disease from owners of affected Akitas:

 "We see much more pemphigus in Akitas and Chow Chow dogs than sebaceous adenitis. Are we alone is this belief? Are there Akita and Chow Chow owners who have suffered with their dogs through this disease who are willing to share with the readers of this newsletter their experiences? We would appreciate  hearing from you. We, at GRF, have long realized that before anything can be done to solve a problem, there must at least be  the admission that the problem is real.