What are the types of Porphyria?

By  ,  National Institute of Health
Jan 11, 2013
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The porphyrias have several different classification systems. The most accurate classification is by the specific enzyme deficiency. Another classification system distinguishes porphyrias that cause neurologic symptoms (acute porphyrias) from those that cause photosensitivity (cutaneous porphyrias). A third classification system is based on whether the excess precursors originate primarily in the liver (hepatic porphyrias) or primarily in the bone marrow (erythropoietic porphyrias). Some porphyrias are classified as more than one of these categories.

Types of Porphyria
Type of Porphyria Deficient Enzyme
Acute Porphyrias
ALAD porphyria delta-aminolevulinic acid dehydratase
acute intermittent porphyria porphobilinogen deaminase
hereditary coproporphyria coproporphyrinogen oxidase
variegate porphyria protoporphyrinogen oxidase
Cutaneous Porphyrias
congenital erythropoietic porphyria uroporphyrinogen III cosynthase
porphyria cutanea tarda uroporphyrinogen decarboxylase (~50% deficiency)
hepatoerythropoietic porphyria uroporphyrinogen decarboxylase (~90% deficiency)
hereditary coproporphyria coproporphyrinogen oxidase
variegate porphyria protoporphyrinogen oxidase
erythropoietic protoporphyria ferrochelatase

The most common type of porphyria overall is porphyria cutanea tarda. In the United States, acute intermittent porphyria is the most common acute porphyria.

 

What are the cutaneous porphyrias?

 

The cutaneous porphyrias affect the skin. People with cutaneous porphyria develop blisters, itching, and swelling of their skin when it is exposed to sunlight. The cutaneous porphyrias include the following types:

 

Read more articles on Porphyria

 

 

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