From the Guidelines
Porphyria Cutanea Tarda (PCT) is the skin rash associated with liver cirrhosis (liver scarring).
Characteristics of PCT
- It is a metabolic disorder due to altered heme biosynthesis pathway 1
- Characterized by low activity of uroporphyrinogen decarboxylase (URO-D) 1
- Presents with typical cutaneous lesions, such as bullae, hyperpigmentation, and erosions at sun-exposed areas 1
Diagnosis of PCT
- Can be suggested by typical cutaneous lesions 1
- Confirmed by the presence of URO-D deficiency and elevated levels of serum and urinary porphyrins 1
- Often associated with liver involvement, which is frequently seen in both HCV and PCT patients 1
Association with Liver Cirrhosis
- PCT is particularly common in patients with liver cirrhosis, especially those with HCV infection 1
- The frequent chronic liver involvement in patients with sporadic PCT suggests a possible pathogenetic link between the two conditions 1
From the Research
Skin Rash Associated with Liver Cirrhosis
The skin rash associated with liver cirrhosis includes:
- Spider angiomas: a common sign observed in patients with liver cirrhosis 2
- Palmar erythema: a common feature in patients with liver disease 3, 4, 5
- Paper money skin: a characteristic skin change in patients with liver cirrhosis 3, 5
- Xanthelasmas: a common feature in patients with liver disease 3
- Pigmentary changes: such as jaundice and melanosis, common in patients with liver cirrhosis 4, 5
- Pruritus: a common symptom in patients with liver disease, often protracted and disabling 3, 4
Other Cutaneous Manifestations
Other cutaneous manifestations associated with liver cirrhosis include:
- Nail changes: such as white nails, clubbing, watch-glass deformity, flat nails, and brittleness 4, 5
- Hair changes: such as loss of axillary and pubic hair 5
- Vascular changes: such as telangiectasias and varicose veins on the abdominal wall ("caput Medusae") 4, 5
- Hormonal changes: such as gynecomastia and female distribution of hair 4, 5