ADULT FORM OF CHRONIC PORPHYRIA WITH CUTANEOUS MANIFESTATIONS

Abstract

The symptoms and laboratory features of chronic or intermediate porphyria may be similar to those of either the acute or the congenital forms, although the course is milder, as a rule. A blistering reaction of the exposed skin of adults, especially on the dorsa of the hands, in response to light, heat, or minor trauma is usually the first manifestation of this disease. The complexion assumes a dusky, bluish-red hue; the conjunctivae are injected, and hypertrichosis may occur. Other symptoms of a vague nature relating to the gastrointestinal system may occur, or nervous and mental disorders may develop. A familial incidence has been noted. The factors which may contribute to the development of the manifest phase of porphyria are not well understood. In so-called acute idiopathic porphyria the barbiturates have long been incriminated. In chronic porphyria a damaging influence on the liver, either by disease, such as cirrhosis, syphilis, diabetes,