Hepatocutaneous Syndrome in Dogs

Hepatocutaneous syndrome (also known as necrolytic migratory erythema, superficial necrolytic dermatitis or metabolic epidermal necrosis) is a rare condition that mainly affects older dogs. The disease is chronic and progressive and in most cases, fatal. While hepatocutaneous syndrome is often linked to diabetes mellitus, the disease may also be connected to a pancreatic, liver or neuroendocrine tumor. Skin lesions are prevalent and are likely the most noticeable symptom, often presenting before lesions on the liver (a severe, degenerative, glycogen-like VH).

Often due to an underlying health condition like diabetes mellitus, hepatocutaneous syndrome is a rare, chronic and progressive disease that often initially presents with skin lesions.

Most dogs with hepatocutaneous syndrome display skin disease; lesions are often found on the muzzle, lower legs or footpads, though the lesions can also be found on the mouth, ear flaps, elbows and genitals of your dog. The lesions will typically have crusting and display ulcerations and in some cases there may be blistering. If footpads are affected they will usually be thickened, possibly have an unpleasant odor and your dog may feel significant pain. Some dogs show signs of systemic illness; your dog may appear listless, have a poor appetite and be experiencing weight loss. Lesions may also be found on your dog’s liver.

Types

There is typically an underlying liver or pancreatic problem in your dog that is leading to his developing hepatocutaneous syndrome. These include:

• Liver tumor
• Pancreatic tumor
• Diabetes mellitus
• Cushing’s disease

In hepatocutaneous syndrome, major liver dysfunction (whether due to a tumor or diabetes mellitus) or a pancreatic tumor cause problems with your dog’s metabolism, leading to a nutritional imbalance and the degeneration of his skin cells. In some dogs, long-term phenobarbital therapy has led to hepatocutaneous syndrome.

Your veterinarian will conduct a physical examination of your dog, and inquire about your dog’s history. It is a good idea to be prepared to share with your veterinarian any changes you have noticed in your dog’s behavior and when you first noticed these changes, as well as when you first noticed the lesions. Your veterinarian will examine the lesions and consider whether your dog has been listless and not eating. Depending upon the physical examination, your veterinarian may request the following:

• Blood work - High liver enzymes and low protein levels are seen in hepatocutaneous syndrome. Blood work may also show increases in ALP, ALT and AST, hyperglycemia, a decrease in plasma amino acid concentrations by about 50% of what is typical and nonregenerative anemia.
• Skin biopsy - The results will lead to further exploration for liver or pancreatic issues.
• Abdominal ultrasound - This will be done to determine if there is a “honeycomb” or “swiss cheese” pattern of the liver caused by liver degeneration, or a pancreatic tumor (pancreatic tumor is a less common cause of hepatocutaneous syndrome).

During diagnosis, should a pancreatic or liver tumor be found, the veterinarian will consider surgically removing it. In cases where the tumor is removed, the skin lesions may resolve for a time. Unfortunately, the tumors will typically spread to other parts of your dog’s body. In cases where the dog is suffering from a condition like diabetes mellitus or Cushing’s syndrome, once the underlying condition is under control, the skin lesions may then resolve.

When your dog is experiencing end stage liver disease, whether at the time of diagnosis or later in the course of the disease, surgery is not an option. At that time, the veterinarian will focus on improving the quality of your dog’s life and help keep him comfortable. Treatment at that time may include:

Protein

Ensuring your dog gets the protein, necessary minerals and enzymes needed through diet, zinc methionine supplements (1.5-2mg/kg/day), vitamins at double the usual dose, antioxidants like vitamin E and SAMe and intravenous amino acid infusions. 

Amino Acids

The amino acids are to be delivered through a jugular vein; for example, Aminosyn 10% crystalline amino acid solution (100 mL has 100 g of amino acids) can be administered intravenously. If skin symptoms don’t resolve, the infusion of amino acids can be repeated 7-10 days later for up to four cycles. If there is no resolution of the skin lesions after four infusions, further infusions will not be useful. 

Skincare

Providing care for the skin lesions through the use of ointments, shampoos and sprays may be recommended. Antifungals or antibiotics for superficial secondary bacterial or fungal infections will be prescribed as needed.

Due to the severity of the disease and the likelihood of an underlying condition being present, follow up appointments will likely be necessary. It is important to work with your veterinarian to ensure the most positive outcome possible for your dog.

Liver disease will often lead to your dog feeling nauseous which may cause him to be uninterested in eating. Efforts to ensure that your dog gets the nutrition he needs are important. To help soften the crusted footpads, you can try foot soaks or moisturizers. Topical anti-yeast sprays or shampoos may be helpful in cases where secondary yeast is affecting your dog. The prognosis is for hepatocutaneous syndrome is guarded to poor and will depend upon the underlying condition.