Overproduction of toxic heme precursors leads to neurovisceral manifestations in porphyria.
Porphyria can affect the autonomic, peripheral, and central nervous systems. Many
drugs and steroids can induce an acute attack of porphyria. Because of its rarity
and ability to mimic other diseases, porphyrias are often overlooked and misdiagnosed.
Hypertension and tachycardia are reported in patients with acute porphyric attack
and are associated with increased sympathetic activity. Here we reported an interesting
case with intermittent cold sweating and shock in acute porphyric attack. A 52-year-old
woman presented with dysuria, cold sweating, intermittent low blood pressure, occasional
ventricular premature beats, and dark-colored urine. Metabolic disease was concerned
in this patient due to the intermittent course of illness. Autonomic neuropathy due
to porphyria was suspected because of dysuria and dark urine. Her symptoms improved
after intravenous glucose injection. Porphyrin level evaluation showed that she had
experienced an acute porphyria attack. This is the first case report of acute porphyria
presenting with intermittent cold sweating and shock. In a sudden onset of shock and
arrhythmia of unknown etiology, metabolic disease such as porphyria should be considered.
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References
- Porphyrias..Lancet. 2005; 365: 241-252
- Posterior reversible encephalopathy syndrome in a patient with acute intermittent porphyria.J Neurol. 2010; 257: 663-664
- Tachycardia and the neuropathy of porphyria.Lancet. 1968; 2: 708-710
- Case records of the Massachusetts General Hospital. Case 20–2008. A 57-year-old woman with abdominal pain and weakness after gastric bypass surgery.N Engl J Med. 2008; 358: 2813-2825
- Acute porphyrias: pathogenesis of neurological manifestations.Semin Liver Dis. 1998; 18: 43-52
- Modern diagnosis and management of the porphyrias.Br J Haematol. 2006; 135: 281-292
- Autonomic neuropathy in acute intermittent porphyria.J Neurol Neurosurg Psychiatry. 1985; 48: 1025-1030
- Variegate porphyria presenting with acute autonomic dysfunction, intussusception and renal infarction.Scand J Gastroenterol. 2004; 39: 500-503
Article Info
Publication History
Published online: October 22, 2012
Accepted:
September 8,
2012
Received in revised form:
September 6,
2012
Received:
August 27,
2012
Footnotes
☆The author received no financial support.
☆☆The article had not been presented.
Identification
Copyright
© 2013 Elsevier Inc. Published by Elsevier Inc. All rights reserved.
