lamellar ichthyosis

Last reviewed 01/2018

• 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.