E80.29
BillableOther porphyria
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 rare inherited metabolic disorder affecting the body's ability to produce heme, causing various types of porphyria with symptoms like abdominal pain, neurological problems, or skin sensitivity.
Coding Tips
- •Specify the type of porphyria when documented (acute intermittent, variegate, etc.) as E80.29 is for unspecified or other types
- •Review clinical documentation for acute attacks versus chronic manifestations to ensure accurate coding
Clinical Significance
Other porphyria captures porphyria types not individually coded, including variegate porphyria (VP), hereditary coproporphyria (HCP), and other rare forms. VP and HCP are dual porphyrias causing both acute neurovisceral attacks AND photosensitive skin disease, making them clinically distinct from both AIP (acute only) and PCT (cutaneous only).
Documentation Requirements
- ✓Specific porphyria type documented (variegate porphyria, hereditary coproporphyria, or other named type)
- ✓Confirmatory biochemical testing: specific porphyrin and porphyrin precursor patterns
- ✓Genetic testing if available (PPOX gene for VP, CPOX gene for HCP)
- ✓Clinical manifestations: acute attacks, skin involvement, or both
- ✓Trigger avoidance plan and safe medication list
- ✓Current management: hemin for acute attacks, sun protection for skin involvement
Commonly Confused Codes
E80.21 — Acute intermittent porphyria: acute attacks only, no skin involvement (code AIP if confirmed)E80.1 — Porphyria cutanea tarda: cutaneous only, no acute attacksE80.20 — Unspecified porphyria: avoid when a specific type like VP or HCP is documentedE80.0 — Hereditary erythropoietic porphyria: different mechanism (erythropoietic, not hepatic)