ABSTRACT

There is considerable variation in clinical expression, but presentation before puberty is uncommon. Affected persons may experience abdominal pain, tachycardia, vomiting, constipation, hypertension, neuropathy, back pain, confusion, bulbar paralysis, psychiatric complaints, fever, urinary frequency, or dysuria. Skin manifestations are similar to those of

porphyria cutanea tarda

and

hereditary coproporphyria

. Neurovisceral manifestations are indistinguishable from those that occur in acute intermittent porphyria and hereditary coproporphyria. Skin manifestations usually occur apart from neurovisceral manifestations. As in acute intermittent porphyria, acute attacks of variegate porphyria can be precipitated by exposure to many drugs, of which barbiturates, sulfonamides, antibiotics, anticonvulsants, and alcohol are the most common. Attacks can also be precipitated by the onset of menses, high progesterone-containing contraceptives, pregnancy, stress, or a sudden decrease in calorie intake.