The main types of porphyria
| Main clinical features | ||||
|---|---|---|---|---|
| Porphyria | Acute neurovisceral attacks | Skin fragility, bullae | Acute photosensitivity | Inheritance |
| In VP acute neurovisceral attacks and skin lesions can occur separately or together; in HCP they usually occur together. | ||||
| *Coinheritance of a severe ferrochelatase defect and a low expression ferrochelatase allele required for clinical expression.5 | ||||
| ALA, 5-aminolevulinate; AR, autosomal recessive; AD, autosomal dominant. | ||||
| ALA dehydratase deficiency porphyria (ALADP) | + | – | – | AR |
| Acute intermittent porphyria (AIP) | + | – | AD | |
| Congenital erythropoietic porphyria (CEP) | – | + | AR | |
| Porphyria cutanea tarda (PCT) | – | + | 15–20% AD | |
| Hereditary coproporphyria (HCP) | + | + | AD | |
| Variegate porphyria (VP) | + | + | AD | |
| Erythropoietic protoporphyria (EPP) | – | – | + | AD* |