Ichthyosis hystrix

This disease is characterised by spiny scales which cover the entire body except the face, genitals, palms and soles.

The only known cases were in Edward Lambert (known as the porcupine man) who was exhibited in front of the Royal Society in London in 1731 and three generations of his descendants.

From the history of the Lambert family the disease appears to have been an autosomal dominant condition.

Symptoms are bilateral hearing loss and spiky hyperkeratotic masses which cover the whole body though the palms and soles are less badly affected.

It can be differentiated from KID syndrome which also has symptoms of deafness and ichthyosis by the different distribution of hyperkeratosis.