Proteus syndrome: Diagnostic criteria, differential diagnosis, and patient evaluation

Biesecker, Leslie G.; Happle, Rudolf; Mulliken, John B.; Weksberg, Rosanna; Graham, John M.; Viljoen, Denis; Cohen, Michael

doi:10.1002/(sici)1096-8628(19990611)84:5<389::aid-ajmg1>3.0.co;2-o

Cited by 389 publications

(329 citation statements)

References 30 publications

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“…The absence of clinically significant arteriovenous shunting distinguishes KTS from Parkes-Weber syndrome. 26 Exact incidence and pathogenesis is still unknown. Diagnosis is purely clinical.…”

Section: Resultsmentioning

confidence: 99%

Klippel-Trenaunay Syndrome: a case report with brief review of literature

Zea¹,

Hanif²,

Habib³

et al. 2010

J Dermatol Case Rep

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Background: Klippel-Tranuanay Syndrome is a rare sporadic disease characterized by clinical triad of capillary malformation; soft tissue and bony hypertrophy; and atypical varicosity. Although this syndrome was first described more than hundred years ago, exact incidence has not been estimated yet. Several theories have been postulated to describe its pathogenesis. Clinical presentation of this syndrome is protean ranging from minimal asymptomatic disease to life threatening bleeding and embolism. Management of this syndrome includes careful diagnosis, prevention and treatment of complications.Main observation: We are presenting a case of Klippel-Tranuanay Syndrome in a 19-year-old male patient presenting with varicosity in lateral aspect of right leg. On careful examination other components of the syndrome were found. Patient was kept on yearly follow up.Conclusion: Usually patients with Klippel-Tranuanay Syndrome present with complication. This case shows that there is also a need for medical solutions in patients who seek advise for cosmetic reasons.

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Section: Resultsmentioning

confidence: 99%

Klippel-Trenaunay Syndrome: a case report with brief review of literature

Zea¹,

Hanif²,

Habib³

et al. 2010

J Dermatol Case Rep

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“…6 Again, these criteria are controversial so it is appropriate to review the history and rationale for their generation. Previous attempts have been made to develop criteria, although these have been supplanted by more recent efforts.…”

Section: Diagnosismentioning

confidence: 99%

“…The meeting conferees reviewed the data on a large number of patients (published and unpublished) and distilled those data into a set of diagnostic criteria. 6 The diagnostic criteria have two categories of attributes; general and specific. The general attributes delineate the nonspecific features of PS by requiring that all patients have a mosaic distribution of the phenotype, are sporadic and the manifestations are progressive in nature.…”

Section: Diagnosismentioning

confidence: 99%

The challenges of Proteus syndrome: diagnosis and management

2006

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“…The absence of clinically significant arteriovenous shunting distinguishes KTS from Parkes-Weber syndrome. 7 The triad of capillary malformations (port wine stain), varicosities or venous malformations and limb hypertrophy have been found to occur in 98%, 72%, and 67% of patients, respectively, in a reported series of 252 patients. 8 The cause of KTS is still unclear.…”

Section: Congenital Venous Diseasesmentioning

confidence: 99%