Cutaneous Porphyria with Porphobilinogenuria: A Review and Report of a Case Treated by Chelation

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

Latent