Clinical classification of porphyrias. Porphyrias can be inherited or (rarely) acquired. With the exception of congenital erythropoietic porphyria (CEP), which is autosomal recessive, all other porphyrias are inherited as autosomal dominant disorders. They invariably result in accumulation and increased excretion of porphyrins and their precursors. Some porphyrias have acute presentations (acute intermittent, variegate, hereditary coproporphyria), whereas others have a chronic, relatively stable presentation (congenital, erythropoietic).
King George III of England had symptoms of abdominal pain, rashes, reddish urine, and psychotic episodes that are consistent with porphyria, although the account is disputed by many. During the period 1955-1959, approximately 4000 people in southeast Anatolia (Turkey) developed porphyria due to the ingestion of hexachlorobenzene (HCB), a fungicide that was added to wheat seedlings.
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