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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

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hepatocutaneous syndrome

Hepatocutaneous Syndrome

Maura St. John

Ultrasound Rotation

Block 19 - 2007

signalment
Signalment

Shelton - 12-year-old MC Shetland Sheepdog

presentation
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
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
Previous Diagnostics
  • ChemPanel
    • 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
Previous Treatments
  • Antibiotics -- Clavamox
    • No response
  • Prednisone
    • Helped paw pads
    • Helped appetite
    • Currently on a 1mg/kg dosage EOD
dermatological examination
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
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
VTH Diagnostics
  • Bile acids tolerance test:
    • Pre: 6.2
    • Post: 15.9
  • Abdominal ultrasound: ….
hyperechoic adrenal nodule
Hyperechoic adrenal nodule
  • Adenoma
  • Hyperplasia
liver
Liver
  • Generally enlarged
  • Diffuse lacy appearance
  • Irregularly shaped hypoechoic nodules/foci separated by thin, hyperechoic tissue
hepatocutaneous syndrome1
HEPATOCUTANEOUS SYNDROME
  • Superficial necrolytic dermatitis
  • Metabolic epidermal necrosis
  • Necrolytic migratory erythema
the swiss cheese liver
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
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.
slide19
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
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
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)