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Acute Intermittent Porphyria (AIP) is probably the most common of the genetic porphyrias. The disorder is expressed clinically after puberty and more commonly in women than in men. Free porphyrins occur only in small amounts in nature. Upon illumination at wavelengths 400nm, in the presence of oxygen, porphyrins generate singlet (unbound) oxygen which causes substantial damage to tissues, cells, subcellular elements, and various biomolecules. Abdominal pain is almost always present and is often the initial symptom of an acute attack. It may be generalized or localized, and in severe cases can be confused with an acute surgical abdomen.
In up to 40% of patients, hypertension may become sustained between acute attacks. Neuropathy is a common feature of AIP. Sensory patchy neuropathy also occurs when motor neuropathy is severe. The course of an acute attack of AIP is highly variable both in individuals and between patients, with attacks lasting from a few days to several months.
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