nonbullous congenital ichthyosis erythroderma

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  • autosomal recessive inheritance
  • incidence of 1 in 300,000
  • generally presents as a 'colloidon baby' - this is followed by generalised erythroderma and scaling
  • the scale in this condition is grey or white; it is light and semi-adherant
  • severe disease may be complicated by fissures on the palms and soles of the feet, partial scarring alopecia and digital contractures
  • ectropions of the eyelids may persist into adult life and can result in exposure keratitis
  • sweating is generally reduced or absent; therefore care is required in avoiding hyperpyrexia
  • the genetic mutation causing this condition is, at present, unknown

Last reviewed 01/2018

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