Acute porphyric attack with cold sweating and low blood pressure

Published:October 22, 2012DOI:
      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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        • Kauppinen R.
        Lancet. 2005; 365: 241-252
        • Kang S.Y.
        • Kang J.H.
        • Choi J.C.
        • et al.
        Posterior reversible encephalopathy syndrome in a patient with acute intermittent porphyria.
        J Neurol. 2010; 257: 663-664
        • Ridley A.
        • Hierons R.
        • Cavanagh J.B.
        Tachycardia and the neuropathy of porphyria.
        Lancet. 1968; 2: 708-710
        • Bonkovsky H.L.
        • Siao P.
        • Roig Z.
        • et al.
        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
        • Meyer U.A.
        • Schuurmans M.M.
        • Lindberg R.L.
        Acute porphyrias: pathogenesis of neurological manifestations.
        Semin Liver Dis. 1998; 18: 43-52
        • Sassa S.
        Modern diagnosis and management of the porphyrias.
        Br J Haematol. 2006; 135: 281-292
        • Laiwah A.C.
        • Macphee G.J.
        • Boyle P.
        • et al.
        Autonomic neuropathy in acute intermittent porphyria.
        J Neurol Neurosurg Psychiatry. 1985; 48: 1025-1030
        • Griffith J.C.
        • Jardine D.L.
        • Bailey W.
        • et al.
        Variegate porphyria presenting with acute autonomic dysfunction, intussusception and renal infarction.
        Scand J Gastroenterol. 2004; 39: 500-503