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Hepatoerythropoietic porphyria

Hepatoerythropoietic porphyria
Uroporphyrinogen III decarboxylase.png
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
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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.



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