[Porphyria variegata]

Report of two patients with porphyria variegata. Several members of the first patient's family had abnormalities of porphyrine metabolism without having manifestations of porphyria. A biochemical screening of family members of PV patients is important because of its prophylactic value. In the second patient's family at least three persons have porphyria-like signs, but not all of them could be seen by us. Diagnosis, pathogenesis and therapy of porphyria variegata are discussed.