Limb hyperplasia: case report of an unusual variant of Klippel-Trenaunay syndrome and review of the literature.
Identifieur interne : 002E28 ( PubMed/Corpus ); précédent : 002E27; suivant : 002E29Limb hyperplasia: case report of an unusual variant of Klippel-Trenaunay syndrome and review of the literature.
Auteurs : Julie K. Gober-Wilcox ; David L. Gardner ; Nancy E. Joste ; Carol L. Clericuzio ; Barrett ZlotoffSource :
- Cutis [ 0011-4162 ] ; 2009.
English descriptors
- KwdEn :
- MESH :
- diagnosis : Klippel-Trenaunay-Weber Syndrome.
- genetics : Klippel-Trenaunay-Weber Syndrome.
- pathology : Klippel-Trenaunay-Weber Syndrome.
- Child, Female, Humans.
Abstract
Klippel-Trenaunay syndrome (KTS) is a rare disorder involving a triad of cutaneous capillary malformations (port-wine stain), varicose veins or venous malformations, and bony or soft tissue hyperplasia of an extremity. It is one of many heterogeneous disorders known as overgrowth syndromes that are characterized by either generalized or localized somatic overgrowth. Overgrowth syndromes each have unique clinical, behavioral, and genetic features, but some of these features overlap, causing diagnostic difficulty. Cutaneous manifestations, however, can be key to distinguishing the various syndromes. We present a patient with an unusual variant of KTS consisting of right upper extremity hyperplasia, lymphedema, and cutaneous and visceral lymphangiomas. We review several closely related syndromes and discuss the differential diagnosis of limb hyperplasia.
PubMed: 19537283
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pubmed:19537283Le document en format XML
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<author><name sortKey="Gober Wilcox, Julie K" sort="Gober Wilcox, Julie K" uniqKey="Gober Wilcox J" first="Julie K" last="Gober-Wilcox">Julie K. Gober-Wilcox</name>
<affiliation><nlm:affiliation>Department of Pathology, University of New Mexico Health Sciences Center, Albuquerque, NM 87131-0001, USA.</nlm:affiliation>
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<author><name sortKey="Gardner, David L" sort="Gardner, David L" uniqKey="Gardner D" first="David L" last="Gardner">David L. Gardner</name>
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<author><name sortKey="Joste, Nancy E" sort="Joste, Nancy E" uniqKey="Joste N" first="Nancy E" last="Joste">Nancy E. Joste</name>
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<author><name sortKey="Clericuzio, Carol L" sort="Clericuzio, Carol L" uniqKey="Clericuzio C" first="Carol L" last="Clericuzio">Carol L. Clericuzio</name>
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<author><name sortKey="Zlotoff, Barrett" sort="Zlotoff, Barrett" uniqKey="Zlotoff B" first="Barrett" last="Zlotoff">Barrett Zlotoff</name>
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<sourceDesc><biblStruct><analytic><title xml:lang="en">Limb hyperplasia: case report of an unusual variant of Klippel-Trenaunay syndrome and review of the literature.</title>
<author><name sortKey="Gober Wilcox, Julie K" sort="Gober Wilcox, Julie K" uniqKey="Gober Wilcox J" first="Julie K" last="Gober-Wilcox">Julie K. Gober-Wilcox</name>
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<author><name sortKey="Gardner, David L" sort="Gardner, David L" uniqKey="Gardner D" first="David L" last="Gardner">David L. Gardner</name>
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<author><name sortKey="Joste, Nancy E" sort="Joste, Nancy E" uniqKey="Joste N" first="Nancy E" last="Joste">Nancy E. Joste</name>
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<author><name sortKey="Clericuzio, Carol L" sort="Clericuzio, Carol L" uniqKey="Clericuzio C" first="Carol L" last="Clericuzio">Carol L. Clericuzio</name>
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<series><title level="j">Cutis</title>
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<term>Klippel-Trenaunay-Weber Syndrome (genetics)</term>
<term>Klippel-Trenaunay-Weber Syndrome (pathology)</term>
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<front><div type="abstract" xml:lang="en">Klippel-Trenaunay syndrome (KTS) is a rare disorder involving a triad of cutaneous capillary malformations (port-wine stain), varicose veins or venous malformations, and bony or soft tissue hyperplasia of an extremity. It is one of many heterogeneous disorders known as overgrowth syndromes that are characterized by either generalized or localized somatic overgrowth. Overgrowth syndromes each have unique clinical, behavioral, and genetic features, but some of these features overlap, causing diagnostic difficulty. Cutaneous manifestations, however, can be key to distinguishing the various syndromes. We present a patient with an unusual variant of KTS consisting of right upper extremity hyperplasia, lymphedema, and cutaneous and visceral lymphangiomas. We review several closely related syndromes and discuss the differential diagnosis of limb hyperplasia.</div>
</front>
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<Abstract><AbstractText>Klippel-Trenaunay syndrome (KTS) is a rare disorder involving a triad of cutaneous capillary malformations (port-wine stain), varicose veins or venous malformations, and bony or soft tissue hyperplasia of an extremity. It is one of many heterogeneous disorders known as overgrowth syndromes that are characterized by either generalized or localized somatic overgrowth. Overgrowth syndromes each have unique clinical, behavioral, and genetic features, but some of these features overlap, causing diagnostic difficulty. Cutaneous manifestations, however, can be key to distinguishing the various syndromes. We present a patient with an unusual variant of KTS consisting of right upper extremity hyperplasia, lymphedema, and cutaneous and visceral lymphangiomas. We review several closely related syndromes and discuss the differential diagnosis of limb hyperplasia.</AbstractText>
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<AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Gober-Wilcox</LastName>
<ForeName>Julie K</ForeName>
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<AffiliationInfo><Affiliation>Department of Pathology, University of New Mexico Health Sciences Center, Albuquerque, NM 87131-0001, USA.</Affiliation>
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<Author ValidYN="Y"><LastName>Joste</LastName>
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<NumberOfReferences>30</NumberOfReferences>
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