Skin symptoms of liver disease

Question 1

Porphyria cutanea tarda

deficiency of what enzyme

Answer 1

Defect or deficiency of Urogen Decarboxylase, UROD

Question 2

Porphyria cutanea tarda

types

Answer 2

both are from UROD deficiency

type 1: only in the liver

type 2: In both liver and RBCs.

Drug induced: Chloroquine, Alcohol, Estrogen,
exacerbates or unmasks latent deficiencyies

Question 3

What lab value is changed in PCT?

Answer 3

Uroporphyrin is found accumulated in urine and in plasma

Question 4

Skin changes of PCT

Answer 4

photosensitive blistering 2-3 days aftyer sun exposure

Periorbital heliotrope rash

Facial hypertrichosis and hyperpigmentation

Coral-red fluorescence of uroporphyrin in urine,
Brown/soda colored urine in normal light.

Generalized fragility of the skin, especially on the hands and arms where small abrasions can cause blisters or erosions.

Question 5

Skin changes associated with liver cirrhosis

Answer 5

Jaundice,

Gynecomastia, galactorrhea - increased estrogen

Hair loss - also from estrogen metabolism

Caput medusae

Spider nevi

Purpuras from coagulation deficiency

Excoriations from scratching due to accumulation of bilirubin in skin.

Duputryens contracture!