March 2003

New Reasons for an Archaic TreatmentPhlebotomy in Sporadic Porphyria Cutanea Tarda

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Copyright 2003 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2003

Arch Dermatol. 2003;139(3):379-380. doi:10.1001/archderm.139.3.379

SINCE ANTIQUITY, blood extraction (phlebotomy) has been empirically and largely used for many conditions and illnesses because it was thought that blood accumulation was the cause or consequence of the trouble. Galen himself proposed this method for different conditions. But for those who are not in the field of medicine, it could be astonishing that this gory and archaic method is still useful in the beginning of the 21st century.

On the other hand, after the description of the first anecdotal cases, the history of porphyrias is now more than 90 years old. The first basic studies of Günther1 recognized the existence of acute, chronic, and congenital forms. In 1937, Waldenstrom2 identified these diseases as being due to porphyrin metabolism abnormalities, and 17 years later, in 1954, the Minneapolis School3 proposed a classification according to the main organ of the abnormal porphyrin overproduction (liver in hepatic porphyrias and bone marrow in erythropoietic ones), which is still valuable. In 1946, Brunsting and Mason4 reported the first evidence of a familial form of the chronic cutaneous disease. Thirty years later, Kushner et al5 showed uroporphyrinogen-decarboxylase deficiency in the liver and red blood cells of some patients with porphyria cutanea tarda (PCT) and found that relatives of patients may also have enzyme deficiency and that it therefore could be transmitted as an autosomal dominant trait. Much more recently, it has been shown that uroporphyrinogen-decarboxylase has a molecular mass of 41 kd, with 367 amino-acid residues, and its gene, located in chromosome 1, has been cloned; it is over 3 kd in length and has 2 initiation sites and 30 exons.6

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