1. Hepatocutaneous Syndrome
Maura St. John
Ultrasound Rotation
Block

2. Signalment
Shelton - 12-year-old MC Shetland Sheepdog

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

4. 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

5. 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

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

7. 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

8. 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.

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

10. Hyperechoic adrenal nodule
Adenoma
Hyperplasia

11. Pancreatic Nodule

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

16. HEPATOCUTANEOUS SYNDROME
Superficial necrolytic dermatitis
Metabolic epidermal necrosis
Necrolytic migratory erythema

17. 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.

18. 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.

19. 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.

20. 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

21. 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)