Variegate Porphyria (VP)

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Variegate porphyria is an autosomal dominant inherited disorder that can be either neurological, cuntaneous, or both. VP neurologic attacks are similar to those of AIP. Drugs often cause the neurologic attacks. The skin symptoms include darkening of the skin and excess hair growth.

Skin Symptoms Acute Attack Symptoms
  • photosensitivity
  • fragility of skin in area exposed to sunlight
  • bullae formation, erosion, and scaring
  • thickening of the skin, excess facial hair growth, and hyperpigmentation may occur
  • abdominal pain
  • hypertension
  • muscular paralysis and sensory disturbance
  • disorientation and psychosis
  • increase heartbeat rate
  • period of prolong disability

Those suffering from VP have an excess level of protoporphyrin and corproporphyrin in the feces at all times and an increase in porphyrins in the urine. This disorder only becomes evident after reaching puberty or may remain unnoticed until   the age of 23. There is a high frequency of VP in South Africa, where the defective gene might have originated from a Dutch settler or his wife, Gerrit Janzs and Ariantje Adriaansse in 1688.

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