Protean elephantsBMJ 1998; 317 doi: https://doi.org/10.1136/bmj.317.7150.89 (Published 04 July 1998) Cite this as: BMJ 1998;317:89
- Jeff Aronson, clinical pharmacologist.
Joseph Merrick, the famous Victorian “elephant man” described by Frederick Treves, has generally been supposed to have suffered from neurofibromatosis. However, in 1986 Tibbles and Cohen (BMJ 1986;293:683-5) suggested that he had instead a rare condition known as Proteus syndrome, and recently this diagnosis has been supported by evidence from x ray examinations and computed tomography of Merrick's remains. Both conditions are hamartoses (from the Greek α ‘μαρτ'∊ν, hamartein, to err or sin), but while neurofibromatosis consists of multiple tumours of nerve cells, Proteus syndrome, a somatic mosaicism, involves abnormal growth of bone and …