ichthyosis hystrix gravior

(redirected from porcupine man syndrome)

ichthyosis hystrix gravior

An autosomal dominant condition (OMIM:146600) characterised by spiny scales covering the entire body (except the face, genitals, palms and soles), the only known cases of which were in Edward Lambert—“The Porcupine Man”, who was exhibited at the Royal Society in London in 1731—and in three generations of his descendants. It is thought to have been a variant of epidermolytic hyperkeratosis.