porcupine man

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porcupine man2016-11-27T19:32:24+00:00

porcupine man 

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Porcupine Man

DERMATOLOGY, GENETICS

A popular term for an über rare autosomal dominant (OMIM:146600) condition 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 three generations of his descendants.

There are no currently known cases; it is thought to have been a variant of epidermolytic hyperkeratosis. 

Synonyms Ichthyosis hystrix gravior, ichthyosis hystrix–Lambert type 

Reference http://www.omim.org/entry/146600  

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