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Porphyria Cutanea Tarda

Reviewed 06/2019
 


BASICS

Porphyria cutanea tarda (PCT) is a condition caused by a defect in the heme biosynthesis pathway in which there is decreased activity of the hepatic uroporphyrinogen decarboxylase (UROD) enzyme...

DIAGNOSIS

The diagnosis of PCT can be established based on the cutaneous manifestations and the characteristic plasma, urinary, and fecal porphyrins. 

HISTORY

  • Development of skin changes with sun exposu...

TREATMENT

  • Therapeutic phlebotomy and hydroxychloroquine are equally efficacious at achieving disease remission. Therapeutic phlebotomy is the preferred treatment for patients with iron overload. Phleb...

ONGOING CARE

Patients with PCT are at increased risk of developing hepatocellular carcinoma especially if they have coexisting liver disease, viral hepatitis, or alcohol use. 

FOLLOW-UP RECOMMENDATIONS

...

REFERENCES

1
Lambrecht RW, Thapar M, Bonkovsky HL. Genetic aspects of porphyria cutanea tarda. Semin Liver Dis.  2007;27(1):99–108. [View Abstract on OvidMedline]
2
Frank J, Poblete-Gutiérrez P. Por...

ADDITIONAL READING

  • Anderson KE, Bloomer JR, Bonkovsky HL, et al. Recommendations for the diagnosis and treatment of the acute porphyrias. Ann Intern Med.  2005;142(6):439–450. [View Abstract on O...

CODES

ICD10

E80.1 Porphyria cutanea tarda 

ICD9

277.1 Disorders of porphyrin metabolism 

SNOMED

  • 61860000 Porphyria cutanea tarda (disorder)

  • 402479002 Sporadic porphyria cutanea tarda (disorder)

  • 59229005 Fami...

CLINICAL PEARLS

  • PCT is a condition that results from a defect in the UROD enzyme in the heme synthesis pathway, causing skin and liver manifestations.

  • Diagnosis should be considered in patients present...

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