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The Proteus syndrome: the Elephant Man diagnosed.
Sir Frederick Treves first showed Joseph Merrick, the famous Elephant Man, to the Pathological Society of London in 1884. A diagnosis of neurofibromatosis was suggested in 1909 and was widely accepted. There is no evidence, however, of café au lait spots or histological proof of neurofibromas. It is also clear that Joseph Merrick's manifestations were much more bizarre than those commonly seen in neurofibromatosis. Evidence indicates that Merrick suffered from the Proteus syndrome and had the following features compatible with this diagnosis: macrocephaly; hyperostosis of the skull; hypertrophy of long bones; and thickened skin and subcutaneous tissues, particularly of the hands and feet, including plantar hyperplasia, lipomas, and other unspecified subcutaneous masses.
- Personal Name as Subject:
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Merrick JC
PMID: 3092979 [PubMed - indexed for MEDLINE]
PMCID: PMC1341524
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Cited by 4 PubMed Central articles
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Non-operative management of a splenic laceration in a patient with the Proteus syndrome.
Ceelen W, De Waele J, Kunnen M, de Hemptinne B.
J Accid Emerg Med. 1997 Mar; 14(2):111-3.
[J Accid Emerg Med. 1997]
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A case illustrating Proteus and Klippel-Trenaunay syndrome overlap.
Sansom JE, Jardine P, Lunt PW, Schutt WH, Kennedy CT.
J R Soc Med. 1993 Aug; 86(8):478-9.
[J R Soc Med. 1993]
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Cranial hemihypertrophy and neurodevelopmental prognosis.
Dean JC, Cole GF, Appleton RE, Burn J, Roberts SA, Donnai D.
J Med Genet. 1990 Mar; 27(3):160-4.
[J Med Genet. 1990]
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