<<snip>>
excellent therapeutic response
<<snip>>
http://qjmed.oxfordjournals.org/cgi/content/abstract/43/1/1
Oxford Journals Medicine QJM: An International Journal of Medicine
Volume 43, Number 1 Pp. 1-24 Q J Med 1974; 43: 1-24
© 1974 Association of Physicians
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research-article
The Treatment of Porphyria Cutanea Tarda by Venesection
C. A. RAMSAY1, I. A. MAGNUS, ADAM TURNBULL* and HARVEY BAKER*
Institute of Dermatology Homerton Grove, London, E9 6BX *The London
Hospital London, El 1BB
Received 7 March 1973 The effect of treatment on 21 patients with
porphyria cutanea tarda is described.
Before treatment the exposed skin showed blistering and fragility,
milia, hirsutes, and changes in pigmentation, with pseudoscleroderma
in some. A history of a heavy alcohol consumption either at
presentation or in the past was obtained from approximately 50 per
cent of the patients whilst 30 per cent admitted to only a moderate
intake of alcohol. No evidence of excess alcohol could be obtained
from the remaining 20 per cent. Plasma iron was raised in 70 per cent
of patients and serial determinations showed that this persisted in 30
per cent.
Porphyrin excretion showed a gross excess of urinary uroporphyrin in
all patients and increased urinary coproporphyrin in 80 per cent. The
changes in faecal porphyrins were less marked and in nearly 60 per
cent were normal.
Liver iron was increased in all patients biopsied. Thirty per cent
showed cirrhosis, the remainder lesser hepatocellular damage. Only one
patient showed completely normal liver-function tests.
Sensitivity of the skin to 400 nm radiation was tested in all but one
of the patients and abnormal reactions obtained in nearly 80 per
cent.
Treatment was by repeated venesections in 16 patients and all save one
showed an excellent response. Urinary uroporphyrin slowly fell to
normal or near normal and this was followed by improvement in the
skin. Spontaneous blistering was the first clinical sign to disappear,
closely followed by cessation of abnormal fragility. Other cutaneous
signs regressed slowly and in most patients the skin returned almost
to normal. One man ceased consumption of alcohol and this was followed
by a complete biochemical and clinical remission. One patient died of
cardiac failure which developed a few months after venesections were
stopped.
Two patients have shown biochemical relapse with a marked rise in
urinary uroporphyrin excretion but with no deterioration in the skin.
A second course of venesections has returned urinary uroporphyrin
excretion to near normal levels in each case.
The way in which venesection produces this excellent therapeutic
response is unknown but several hypotheses are discussed, e.g.
depletion of iron in the hepatic cell.
It is suggested that venesection is the treatment of choice for
porphyria cutanea tarda.
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1Dr. Ramsay is in receipt of a grant from The Medical Research
Council.
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After treatment,MRI lesions showed marked resolution of signal
abnormalities.
Quoted post said:J Korean Neurol Assoc. 1998 Apr;16(2):241-244. Korean.
A Case of Porphyria withreversible MRIfindings.
Kang SY, Ma HI, Kwak KH, Lee SA.
Dept. of Neurology, Ulsan University, College of Medicine, Asan
Medical Center.
The porphyrias are inherited or acquired disorders of specific enzymes
in the heme biosynthetic pathway. Porphyrias have diverse neurologic
manifestations with unknown mechanism. Some authors have proposed
ischemic vasculopathy as a cause of neurologic deficits, which was
supported byreversibleMRIlesions in porphyrias. We report a rare
case withreversibleMRIfindings in porphyria. A 27-year-old woman
was presented with seizures and hallucinations.MRIrevealed multiple
lesions of both hemispheres. After treatment,MRIlesions showed
marked resolution of signal abnormalities.
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