PORPHYRIC BULLOUS DERMATOSIS

Abstract

PORPHYRIA, a peculiar disease of metabolism in which port-wine-colored urine, containing quantities of uroporphyrin, is excreted, has been reported associated with a number of different clinical manifestations. All normal urine of human beings contain, minute quantities of coproporphyrin. Various normal values for the average twenty-four hour excretion of coproporphyrin have been given by authors using different technics, but the maximal excretion of over 100 specimens previously examined by one of us (I. Z.) represented 3 micrograms per kilogram of body weight.¹ Increased excretion of coproporphyrin, or porphyrinuria, occurs in hepatic diseases, hemopoietic abnormalities and febrile disorders.² On the other hand, uroporphyrin is not found in normal urine nor is it specifically found in the aforementioned pathologic states. Porphyria is characterized by the excretion of the abnormal porphyrin, uroporphyrin, usually in such quantities that the urine is reddish brown. Many classifications of the porphyrias have been made. Günther's³