clinical features

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Symptomatic variegate porphyria may manifest with (1):

  • skin lesions alone (about 60% cases)
  • acute attacks alone (20% cases)
  • both (20% cases)

The clinical features of variegate porphyria show some sexual differences with females presenting with acute attacks of typical of acute intermittent porphyria and males having the cutaneous lesions of porphyria cutanea tarda.

The skin lesions of VP present in the second and third decades compared to the fourth or fifth in PCT. They include bullae, erosions or ulcers following minor trauma of light exposed skin. There may be milia. Chronic changes include crusting, depigmented scarring and hypertrichosis.

There is a history of acute episodes of abdominal pain, nausea, vomiting, paralysis, constipation, urinary frequency, dysuria, tachycardia, hypertension (2), seizures and neuropsychiatric manifestations of stupor and coma.

Reference:

Last reviewed 11/2021

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