Hepatocutaneous Syndrome

Also known as: Necrolytic Migratory Erythema — Metabolic Epidermal Necrosis — Superficial Necrolytic Dermatitis

Introduction

This is an uncommon metabolic skin disease of dogs, in which a vacuolar hepatopathy is associated with an ulcerative necrolytic dermatitis.

The aetiology is unknown, but hepatic disease, particularly vacuolar degeneration or cirrhosis, or pancreatic tumours producing glucagon (which is the syndrome seen in humans) are present. Some dogs also have diabetes mellitus. The link between systemic disease and the skin disorder is unclear but may reflect low plasma levels of amino acids. Whether the changes in the liver or pancreas are a cause or an effect of the hypoaminoacidaemia is unknown.

The skin disease may precede or occur at the same time as signs of internal disease.

The disease tends to occur in older animals, with an average age of 10.5 years.

Clinical Signs

The most common presentation is a skin disease involving: erythema, crusting, exudation and ulceration involving the footpads, pressure points on the trunk and limbs, muzzle, perineum and periocular areas. The trunk is also sometimes affected.

Dogs presenting only with liver disease will show lethargy and anorexia. Skin disease will then develop within a few weeks.

Lameness due to footpad disease may be the only presenting sign.

Diagnosis

The condition is often misdiagnosed as an autoimmune disorder due to the similar clinical signs. Pemphigus foliaceus, SLE and drug eruptions should be ruled out.

Haematology and biochemistry may reveal: increased ALT, AST and ALP, hyperglycaemia without ketoacidosis, Regenerative and Non-regenerative Anaemias|non-regenerative or mildly regenerative anaemia]], hypoalbuminaemia, severe hypoaminoacidaemia, elevated bile acids.

Abdominal ultrasonography may reveal diffuse hepatic disease (a 'honeycomb pattern') or a pancreatic tumour and its metastases.

Liver cyotology may show severe, vacuolar degeneration of hepatocytes.

Skin biopsies are the most consistent diagnostic test. They show a characteristic pattern of parakeratosis, upper epidermal pallor with oedema, and a superficial dermal infiltrate of mononuclear cells.

Treatment

There is no specific treatment for the skin signs, however amino acid supplementation is usually performed, either by increased protein in the diet or by administering amino acid mixtures parenterally.

If there is no response after 4-5 treatments it is unlikely that the patient will ever respond.

If clinical signs resolve, then amino acid therapy should be administered as needed.

Steroids sometimes improve the skin lesions but are contraindicated because of the metabolic disease.

Some skin signs may resolve following treatment of the underlying metabolic disease, such as diabetes mellitus. Unfortunately, the hepatic or pancreatic disease often associated is usually serious and irreversible, and therefore the skin lesions tend to progress or the animal will die.

The prognosis is therefore often grave.

Hepatocutaneous Syndrome Learning Resources
Flashcards Test your knowledge using flashcard type questions	Small Animal Abdominal and Metabolic Disorders Q&A 04

References

Bond, R. (2010) Miscellaneous canine diseases RVC student notes

Schaer, M. (2010) Clinical Medicine of the dog and cat Manson Publishing

Moriello, K. (2005) Self-assessment colour review of small animal dermatology Manson Publishing