Hepatocutaneous Syndrome Maura St. John Ultrasound Rotation Block 19 - 2007

Signalment Shelton - 12-year-old MC Shetland Sheepdog

Presentation Seen by Dermatology Evaluation of skin problems mainly affecting the paws & paw pads beginning in September 2006 as hardening, enlarged paw pads

Clinical Signs beginning in September 2006 as hardening, enlarged paw pads lethargy & inappetence, attributed to the pain associated with the paw pads by the rDVM

Previous Diagnostics ChemPanel Biopsy Increased liver enzymes (ALP, GGT, ALT, AST) Biopsy Moderate parakeratosis with irregular epidermal hyperplasia & increased basophilia Suggestive of superficial necrolytic dermatitis with secondary pyoderma

Previous Treatments Antibiotics -- Clavamox Prednisone No response Helped paw pads Helped appetite Currently on a 1mg/kg dosage EOD

Dermatological Examination All four paw pads are hyperkeratinized with excessive scaling/crusting, especially around the edges of the paws Few fissures on the paw pads & interdigital accumulation of the hyperkeratoic material is obvious on all paws

Physical Examination T 101.7, P 110, R 30. PLN: Left popliteal LN mildly enlarged EENT: Cataracts OU, diminished PLRs OU. GI/GU: Unremarkable. MSN: Unremarkable. CV/P: Unremarkable.

VTH Diagnostics Bile acids tolerance test: Abdominal ultrasound: …. Pre: 6.2 Post: 15.9 Abdominal ultrasound: ….

Hyperechoic adrenal nodule Adenoma Hyperplasia

Pancreatic Nodule

Liver Generally enlarged Diffuse lacy appearance Irregularly shaped hypoechoic nodules/foci separated by thin, hyperechoic tissue

HEPATOCUTANEOUS SYNDROME Superficial necrolytic dermatitis Metabolic epidermal necrosis Necrolytic migratory erythema

The “Swiss Cheese” Liver Ultrasound exam may demonstrate a pancreatic tumor or a honeycomb or Swiss cheese appearance to the liver because a hyperechoic network surrounds hypoechoic areas of parenchyma. Histologically, the hypoechoic regions correspond to distinct regenerative nodules bounded by severely vacuolated (fat-laden) hepatocytes, numerous bile ductules, and a network of reticulin and fine collagen fibers representing remnants of collapsed hepatic lobules.

So, what causes this? The pathogenesis is not completely understood. It is believed that deficiencies in certain nutrients (e.g., amino acids, biotin, essential fatty acids, zinc) probably cause keratinocyte degeneration. The most common pathologic association is liver disease, although diabetes mellitus and pancreatic tumors have also been reported. Chronic administration of phenobarbital may also be a risk factor for the development of SND.

FNA A fine-needle aspirate or ultrasound-guided biopsy sample from the liver may be either helpful or misleading. vacuolar hepatopathy ballooning degeneration This may be interpreted as fatty infiltration or steroid hepatopathy, especially if the animal has been on prior glucocorticoid therapy for the presenting clinical complaints.

Specific Treatment Amino Acids 8.5% without electrolytes Central venous catheter infused slowly (80mL/hour) Repeated every 2 weeks until resolution of signs Then, as needed

Supportive Treatments Vitamin K 1mg/kg q 7 days subcutis Vitamin B 50mcg/kg twice in 2-week interval Then continued monthly Zinc gluconate -- 5mg/kg SID SAM-e -- 18mg/kg SID DIET: high in essential fatty acids (Eukanuba FP)