Table 1B.

Summary of major clinical features of the porphyrias.

Type of PorphyriaNeurovisceralClinical Manifestation CutaneousLiver Damage
The porphyrias are listed with respect to their dominant symptoms with neurovisceral or cutaneous manifestations and the presence of potential liver damage. 
Reprinted from
Chemmanur AT, Bonkovsky HL. Hepatic porphyrias: diagnosis and management.
Clin Liver Dis
.
2004
;
8
:
807
–838, with permission from Elsevier.
 
Abbreviations: ADP, ALA dehydratase deficiency porphyria; AIP, acute intermittent porphyria; ALA, 5-aminolevulinate; CEP, congenital erythorpoietic porphyria; EPP, erythorpoietic protoporphyria; HCP, hereditary coproporphyria; HEP, hepatoerythrocytic porphyria; PCT, porphyria cutanea tarda; VP, variegate porphyria 
* EPP with end-stage liver disease, especially just after liver transplantation, may rarely be associated with neurovisceral manifestations. 
ADP Yes No No 
AIP Yes No No 
HCP Yes Yes (bullae, fragility) No 
VP Yes Yes (bullae, fragility) No 
PCT No Yes (bullae, fragility) Yes 
HEP +/− Yes (bullae, fragility) Yes 
CEP No Yes (bullae, fragility) Occasional 
EPP Rarely* Yes (urticaria, erythema) Yes (10%) 
Type of PorphyriaNeurovisceralClinical Manifestation CutaneousLiver Damage
The porphyrias are listed with respect to their dominant symptoms with neurovisceral or cutaneous manifestations and the presence of potential liver damage. 
Reprinted from
Chemmanur AT, Bonkovsky HL. Hepatic porphyrias: diagnosis and management.
Clin Liver Dis
.
2004
;
8
:
807
–838, with permission from Elsevier.
 
Abbreviations: ADP, ALA dehydratase deficiency porphyria; AIP, acute intermittent porphyria; ALA, 5-aminolevulinate; CEP, congenital erythorpoietic porphyria; EPP, erythorpoietic protoporphyria; HCP, hereditary coproporphyria; HEP, hepatoerythrocytic porphyria; PCT, porphyria cutanea tarda; VP, variegate porphyria 
* EPP with end-stage liver disease, especially just after liver transplantation, may rarely be associated with neurovisceral manifestations. 
ADP Yes No No 
AIP Yes No No 
HCP Yes Yes (bullae, fragility) No 
VP Yes Yes (bullae, fragility) No 
PCT No Yes (bullae, fragility) Yes 
HEP +/− Yes (bullae, fragility) Yes 
CEP No Yes (bullae, fragility) Occasional 
EPP Rarely* Yes (urticaria, erythema) Yes (10%) 
Close Modal

or Create an Account

Close Modal
Close Modal