. Author manuscript; available in PMC: 2020 May 16.

Published in final edited form as: Genet Med. 2019 May 10;21(11):2605–2613. doi: 10.1038/s41436-019-0537-7

Table 5.

Key porphyrin biochemistry findings characteristic of active acute versus cutaneous presentation of AHPs

	During acute attacks				Cutaneous manifestations
Porphyria	Urine ALA/PBG	Total fecal porphyrins	FCR	Plasma scan^a (nm)	Urine ALA/PBG	Total fecal porphyrins	FCR	Plasma scan^a (nm)
AIP	Highly increased > 4-fold URL	±Normal	<1.5	615–620	Not applicable
HCP	Highly increased > 4-fold URL	Highly increased (Copro III)	>1.5	615–620	±Normal	Highly increased (Copro III)	>1.5	615–620
VP	Highly increased > 4-fold URL	Highly increased (Proto IX & Copro III)	>1.5	625–628	±Normal	Highly increased (Proto IX & Copro III)	>1.5	625–628

ALA 5-aminolaevulinic acid, Copro III coproporphyrin III isomer, FCR fecal coproporphyrin III:I isomer ratio, PBG porphobilinogen, Proto IX protoporphyrin IX, URL upper reference limit.

Plasma porphyrin fluorescence scan emission wavelength.