Mucinosis

This summarizes my knowledge of and personal experiences with idiopathic mucinosis. It is supplemented by information that many Shar-Pei owners sent to me on how their dogs suffered from the complex symptoms of idiopathic mucinosis.

Our Shar-Pei bitch, Pief / Caipiriha, was struck with mucinosis in September 2007. That very month, her upper and lower eyelids also had to be operated for entropion. Pief was born on June 23, 2005, stems from the C-litter of Chaitan’s Kennel, and her breeder is Uwe Lenk!  

Shar-Pei dogs suffer from a genetic disorder that affects their skin folds about the head, eyes, and forehead, and the deposits in the flews and carpal ligaments. This hereditary skin disease called idiopathic mucinosis, often results in excessive mucin, causing the skin to form vesicles about the throat, stomach, and axillae. The disease may also affect the mucous membrane of the nose and pharyngeal space.

Shar-Pei dogs are prone to idiopathic mucinosis, which is passed down particular breeding lines. Many clinics have identified this specific illness through skin biopsies. According to the latest information I have, idiopathic mucinosis is one of the most common breeding diseases among the Shar-Pei in Germany – and the trend is worsening.

If the vesicles are opened or if they rupture, they ooze out the clear, light to honey-colored, sticky fluid mucin containing protein. The vesicles can last a very long time and also become huge, especially around the neck. Mostly young dogs are affected, but adults also tend to suffer from this disease. Diagnosis involves a mucinosis test for histological evidence of skin disease. Accordingly, a vesicle is punctured and if the dog has mucinosis, the inflammation secretes out mucin.

If the dog scratches and ruptures the bubbles or vesicles, or this accidentally happens from something like the rubbing of a collar, and the vesicles become inflamed, you should immediately apply a non-corticosteroid anti-inflammatory ointment on the wounds. If dogs are treated with cortisone, they tend to lose their fatty tissue and mucin deposits in the skin folds about the head (eyes, forehead), flews, and carpal ligaments. Besides, cortisone also dehydrates the tissue, and these side effects can lead to further problems. Normally, Shar-Pei dogs with idiopathic mucinosis are not treated, since the symptoms tend to disappear on their own in time.

The condition can be serious if a Shar-Pei dog with idiopathic mucinosis comes down simultaneously with another familial illness. That is precisely what happened to our Pief. Her eyelids had to be operated immediately, because of the genetic disease, entropion. During the eyelid operation and the subsequent recovery period, the excessive production of mucin turned out to be quite problematic. Since, the eyelid tissue was very soft after the operation and mucin oozed out of the sutures, the risk of an infection was very high and the scabbing required a longer time to heal.

I believe that idiopathic mucinosis is triggered by the aftereffects of a metabolic disorder, and changing the diet is often helpful. Mucinosis vesicles could still arise from hypothyroidism (underactive thyroid), IgA deficiency, LE (lupus erythematosus), or dermatomyositis. Following a diagnosis of mucinosis, the vet should clarify if the formation of vesicles is really idiopathic. If so, the therapy should naturally focus on treating the underlying disease.

Idiopathic diseases: These are diseases of unknown cause, in which the symptoms indicate the disease, but cannot be traced to any particular origin.

IgA deficiency: IgA is an antibody class of protein or immunoglobulin. This condition is termed selective IgA deficiency, since normal amounts of other immunoglobulins like IgM and IgG are present but the body lacks the particular antibodies needed primarily to fight infections in mucous membranes or bodily secretions. An IgA deficiency is considered one of the most common immunodisorders.

LE (lupus erythematosus): This illness surfaces as chronic inflammations caused by a disorder of the immunoregulatory mechanism. LE is related to collagenosis, since the antibodies erroneously produced by the immune system also attack collagen containing connective tissue. Such tissue is found in different bodily structures like the skin.

Dermatomyositis: This disease is similar to systemic LE, but it appears as an infestation of the skin and musculature. 

People have found that the following provides relief for Shar-Pei with general skin diseases. Perhaps these will also help treat the formation of vesicles in our dogs suffering from the hereditary skin disease, idiopathic mucinosis:

1.    Administer an anti-inflammatory ointment on the wounds and ruptured vesicles.

2.    Change to a balanced diet with the following ingredients: poultry, puffed whole grain rice, puffed whole grain corn, wild game rumen, wild game bones, sea fish, corn germ, fresh green herbs, blueberries, egg yolk, cold-pressed vegetable oil mixture, wild salmon oil, Jerusalem artichokes, algae mixture, trace elements, and vitamins; about 360 kcal/100 g, protein around 20%, and NO carrots, since these turn the coat red.

3.    Bathe the dog in seawater prepared as follows. This non-binding recommendation is from Rüdiger Hanisch, http://www.sharpei-forum.de/, who also holds the copyright.

Seawater recipe:

a.    1 plastic container (10 liters) of distilled water (neutral pH)
b.    1 small sack of sea salt (available in a zoo shop with a good aquarium section, alternatively buy Dead Sea salt from a drugstore)
c.    2 plastic buckets, 10 liters ea.

First treatment: stir and mix 216 g of sea salt in 8 liters of distilled water (27g/liter); set aside for two days, pour several times from one bucket into the other from a height of about 1.5 m so that a lot of air is entrained in the fluid. Alternatively, use a pneumatic pump for an aquarium to aerate the fluid for two days.

Wet and wash the dog thoroughly with this seawater; after about an hour, rinse the dog with normal lukewarm tap water.

Subsequent treatments: increase the dosage of sea salt to 33.3 g/liter of distilled water.

I have not evaluated the statements about products and health conditions. Hence, any information on diagnostics, therapies, and medications is not intended as a personal recommendation of any kind whatsoever. This statement also covers the recommendation made by Rüdiger Hanisch.

Never give your dog any medicines without consulting your vet.

If you are interested in delving further into idiopathic mucinosis, go to the following helpful links:

Mast Cell Density and Subtypes in the Skin of Shar Pei Dogs with Cutaneous Mucinosis:
http://www.blackwell-synergy.com/doi/abs/10.1046/j.1439-0442.1999.00220.x

Cutaneous mucinosis and mastocytosis in a shar-pei 
http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1539873


Hanspeter Kobold
Bremen, October 2007

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