Transcript Hepatocutaneous Syndrome

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
 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
 No response
• Prednisone
 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:
 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 ultrasoundguided 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)