PORPHYRIA WITH EPIDERMOLYSIS BULLOSA

Abstract

Bullous eruptions simulating epidermolysis bullosa, in which the exposed surfaces are sensitive to light, occur in association with porphyria and may represent the presenting symptoms of the disorder. Turner and Obermayer¹ described such a case of epidermolysis bullosa, with melanosis and hypertrichosis as well, and in a detailed survey of the literature and a discussion of the chemistry of the problem, listed 14 similar cases. They also reviewed 200 cases of hydroa and found that in 86 instances of the more severe type, hydroa vacciniforme, there was associated porphyria. Porphyria is recognized as that rare, familial, metabolic fault in which abnormal kinds and amounts of porphyrins, especially uroporphyrin, are excreted in the urine and feces. The disease may be latent or asymptomatic for years, with or without the characteristic coloration of the urine. Surveys of families in which porphyria occurs may disclose the presence of uroporphyrin or the colorless