nonbullous congenital ichthyosis erythroderma
Last reviewed 01/2018
- 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