E80.1
BillablePorphyria cutanea tarda
HCC Category Mapping
V28HCC 50 — Glycogen/Amino-Acid/Other Metabolic Disorders
0.289V24HCC 23 — Other Significant Endocrine and Metabolic Disorders
0.230ESRDHCC 23 — Other Significant Endocrine and Metabolic Disorders
0.000RxHCCHCC 43 — Other Significant Endocrine and Metabolic Disorders
0.000What This Code Means
A porphyria disorder causing blistering and fragile skin on sun-exposed areas, often triggered by liver disease or certain medications.
Coding Tips
- •Document triggering factors such as hepatitis C, HIV, or medication use
- •Code associated liver disease separately if present
Clinical Significance
Porphyria cutanea tarda (PCT) is the most common porphyria, characterized by photosensitive blistering on sun-exposed skin, skin fragility, hypertrichosis, and hyperpigmentation. It is often associated with hepatic iron overload, hepatitis C, alcohol use, and estrogen exposure, and is the only porphyria primarily treated by phlebotomy to reduce iron stores.
Documentation Requirements
- ✓Provider diagnosis of porphyria cutanea tarda
- ✓Elevated urine and plasma uroporphyrins with characteristic porphyrin pattern
- ✓Skin findings: blistering, skin fragility, hypertrichosis, scarring on sun-exposed areas (hands, forearms, face)
- ✓Iron studies: ferritin level, hepatic iron assessment
- ✓Assessment for associated conditions: hepatitis C, alcohol use, hemochromatosis, HIV, estrogen use
- ✓Treatment plan: therapeutic phlebotomy, low-dose hydroxychloroquine, or iron chelation
Commonly Confused Codes
E80.0 — Hereditary erythropoietic porphyria: much rarer and more severe, with childhood onsetE80.21 — Acute intermittent porphyria: neurovisceral attacks, NOT skin blisteringE80.29 — Other porphyria: for porphyria types like variegate porphyria or hereditary coproporphyriaL10.9 — Pemphigus, unspecified: autoimmune blistering disease that may mimic PCTL12.0 — Bullous pemphigoid: another blistering condition that may be confused with PCT on sun-exposed skin
Code Hierarchy
└E80Disorders of porphyrin and bilirubin metabolism└E80.1Porphyria cutanea tarda
└E80.1Porphyria cutanea tarda