Summary of major clinical features of the porphyrias.
Type of Porphyria . | Neurovisceral . | Clinical Manifestation Cutaneous . | Liver 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 Porphyria . | Neurovisceral . | Clinical Manifestation Cutaneous . | Liver 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%) |