| Hepatoerythropoietic porphyria | |
|---|---|
| UroD drawn from PDB: 1URO. | |
| Classification and external resources | |
| ICD-10 | E80.2 (ILDS E80.282) |
| ICD-9-CM | 277.1 |
| OMIM | 176100 |
| DiseasesDB | 29123 |
| MeSH | D017121 |
Hepatoerythropoietic porphyria is a very rare form of hepatic porphyria caused by a disorder in both genes which code Uroporphyrinogen III decarboxylase (UROD).
It has a similar presentation to porphyria cutanea tarda (PCT), but with earlier onset. In classifications which define PCT type 1 as "sporadic" and PCT type 2 as "familial", hepatoerythropoietic porphyria is more similar to type 2.