An Interesting Case of Acute Intermittent Porphyria Precipitated During Premenstrual Phase

Abstract

Acute intermittent porphyria is one of the rare, autosomal dominant, hereditary hepatic porphyria due to deficiency of enzyme Hydroxyl Methyl Bilane synthase (HMB synthase) in the haem synthesis pathway. The condition is characterized by acute abdominal pain and neuropsychiatric manifestations. One of the precipitating causes of AIP is menstruation due to high levels of progesterone. We report an interesting case of AIP precipitated during each cycle of menstruation.