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A RARE CASE OF VARIEGATE PORPHYRIA - A CASE REPORT

S Jaya, Romy W MARSHNIL

Abstract


Variegate porphyria is a rare autosomal domi-nant disorder which occurs due to a deficiency of the enzyme protoporphyrinogen oxidase (1,2). The name variegate was first coined by Dean and Barnes (3).This disease was first described in south African white population. The disease fre-quency is 3 cases per 1000 persons. The disease shows a varied presentation which includes neu-rovisceral, cutaneous manifestations or both and hence its name.

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An Initiative of The Tamil Nadu Dr M.G.R. Medical University