Cutaneous Porphyria with Porphobilinogenuria
- 1 May 1958
- journal article
- case report
- Published by American Medical Association (AMA) in A.M.A. Archives of Dermatology
- Vol. 77 (5) , 559-567
- https://doi.org/10.1001/archderm.1958.01560050069012
Abstract
Since Günther's1 clinical study of the now-famous case of Petry, a congenital porphyric (erythropoietica of Watson2), and Hans Fischer's3 biochemical investigation of this patient in 1915, the term porphyria has grown to embrace a group of deceptively different, yet similar, syndromes. It is perhaps best regarded as a group of inborn errors of porphyrin metabolism which find expression in the adult most commonly as an acute, often fatal, abdominal-neurological disorder or, less commonly, as a more chronic photosensitive cutaneous disorder. Increasing knowledge of the biochemistry of the porphyrins and the frequent merging of one syndrome into the other have offered a perplexing clinical problem. The classifications of Günther,1 Waldenström,4 Kark,5 and Watson2 bear witness to this problem, the latter being widely accepted in this country. Watson's classification is as follows: Porphyria Erythropoietica Porphyria Hepatica Acute Intermittent Cutanea tarda Mixed LatentKeywords
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