PREDICTABLE AND UNPREDICTABLE HAZARDS OF ERYTHROPOIETIC PROTOPORPHYRIA

A female patient with a long history of erythropoietic protoporphyria developed progressive rapid derangement of hepatic function requiring urgent orthotopic liver transplantation. In the immediate post-operative period the patient developed unusual and serious complications, including a generalized axonal neuropathy, intestinal ulceration and a large second-degree phototoxic burn of the abdominal wall. Recovery was achieved only after prolonged intensive hospital care and rehabilitation. Attention is drawn to the need for protecting such patients from phototoxic injury during prolonged surgery and the importance of rapid transfer to a specialized unit at the earliest sign of hepatic failure.