Comparison of the major features of the four acute porphyrias

	Deficient enzyme (step in heme synthesis)	Inheritance	Neurovisceral manifestations	Blistering skin lesions
ALA dehydratase porphyria (ADP)	ALA dehydratase (2)	Autosomal recessive	Yes	No
Acute intermittent porphyria (AIP)	PBG deaminase (3)	Autosomal dominant, variable penetrance	Yes	No*
Hereditary coproporphyria (HCP)	Coproporphyrinogen oxidase (6)	Autosomal dominant, variable penetrance	Yes	Uncommon
Variegate porphyria (VP)	Protoporphyrinogen oxidase (7)	Autosomal dominant, variable penetrance	Yes	Common

Refer to UpToDate topics on porphyria for details.

ALA: 5-aminolevulinic acid (delta-aminolevulinic acid); PBG: porphobilinogen.
* A rare exception is patients with advanced renal failure, who may develop elevations in plasma porphyrins that produce blistering skin lesions.

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Comparison of the major features of the four acute porphyrias