Porphyria cutanea tarda

This code represents porphyria cutanea tarda (PCT), a chronic blistering skin condition caused by a deficiency in the uroporphyrinogen decarboxylase enzyme, leading to accumulation of porphyrins in the skin. Patients typically present with photosensitivity, fragile skin, bullae, hyperpigmentation, and hypertrichosis, often exacerbated by sun exposure.

Use this code for patients diagnosed with porphyria cutanea tarda, confirmed by elevated porphyrin levels in urine, stool, or plasma. This diagnosis is often associated with underlying liver disease, alcohol use, or iron overload, which should also be coded. Documentation should clearly state the diagnosis of PCT.

• Differentiate from other types of porphyria; this code is specific to porphyria cutanea tarda.
• Always code any associated conditions, such as chronic liver disease (e.g., alcoholic liver disease, hepatitis C), hemochromatosis, or HIV, as these are common triggers or comorbidities.
• Ensure documentation specifies "cutanea tarda" to avoid confusion with other porphyrias.