Abdominal pain caused by AIP is neuropathic in origin and is severe and diffuse. It usually lasts for hours or days, beginning gradually and escalating in severity. It is rarely accompanied by peritoneal signs, fever, or leukocytosis.1,2
Signs and symptoms:
a deeper look
- Mild sensory
Urine may become discolored (ranging from pink to darker red to brown to nearly black) when exposed to air and light due to porphyrin or porphobilin formation.2
Paresis may occur early or late in a severe attack. Muscle weakness usually begins proximally in the upper extremities and may resemble Guillain-Barré syndrome, but it may be focal and involve cranial nerves.1,3
Convulsions may be caused by a central neurologic manifestation of porphyria or by hyponatremia.1
Neurologic pain may begin in the chest or back and move to the abdomen. Extremity pain indicates involvement of sensory nerves.1
Mild sensory changes often accompany the predominantly motor neuropathy, often in a “bathing trunk” distribution.4
Mental symptoms may range from minor behavioral changes to agitation, confusion, hallucinations, and depression. A period of minor behavioral changes, such as anxiety, irritability, restlessness, and insomnia, may precede an acute attack.1,4
Atypical presentations can occur. Conversely, not all symptoms in porphyric patients may be due to AIP. In addition, symptoms of AIP may vary considerably, even in the same patient during different episodes. 1,5,6
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- Anderson KE, Bloomer JR, Bonkovsky HL, Kushner JP, Pierach CA, Pimstone NR, Desnick RJ. Recommendations for the diagnosis and treatment of the acute porphyrias. Ann Intern Med. 2005;142:439-450.
- Besur S, Hou W, Schmeltzer P, Bonkovsky HL. Clinically important features of porphyrin and heme metabolism and the porphyrias. Metabolites. 2014;4:977-1006.
- Ventura P, Cappellini MD, Biolcati G, Guida CC, Rocchi E. A challenging diagnosis for potential fatal diseases: Recommendations for diagnosing acute porphyrias. Eur J Intern Med. 2014;25:497-505.
- Ventura P, Cappellini MD, Rocchi E. The acute porphyrias: A diagnostic and therapeutic challenge in internal and emergency medicine. Intern Emerg Med. 2009;4:297-308.
- Gonzalez-Arriaza HL, Bostwick JM. Acute porphyrias: A case report and review. Am J Psych. 2003;160(3);450-458.
- Thadani H, Deacon A, Peters T. Diagnosis and management of porphyria. BMJ. 2000;320(7250);1647-1651.