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- lamellar ichthyosis is inherited as an autosomal recessive with an estimated incidence of less than 1 per 300,000
- usually it starts at birth or within the first 3 months. It begins as a generalised erythema and subsequently the skin becomes thickened and scaly. The entire cutaneous area may become scaly - including flexures, palms and soles
- the scales are large and parchment-like. They may be verrucous especially around the joints
- additional features in some patients include palmoplantar hyperkeratosis, ectropion and scarring alopecia
- sweating may be abnormal and the patient hyperpyrexial. Skin infections are common
- the genetic mutation causing lamellar ichthyosis is, at present, unknown
It may present as a "collodion baby" - premature, and with shiny skin which feels hard and rigid.
Last reviewed 01/2018