Skin-Related complications of Klippel-Trenaunay Syndrome: a retrospective review of 410 patients.
Journal
Journal of the European Academy of Dermatology and Venereology : JEADV
ISSN: 1468-3083
Titre abrégé: J Eur Acad Dermatol Venereol
Pays: England
ID NLM: 9216037
Informations de publication
Date de publication:
Feb 2021
Feb 2021
Historique:
received:
21
02
2020
accepted:
29
09
2020
pubmed:
19
10
2020
medline:
15
5
2021
entrez:
18
10
2020
Statut:
ppublish
Résumé
Little is known about skin-related complications in Klippel-Trenaunay syndrome (KTS), a complex vascular anomaly defined by capillary malformation (CM), venous malformation (VM) ± lymphatic malformation (LM) and limb overgrowth. Reported skin-related complications of KTS include ulceration, vascular ectasias (blebs), bleeding and infection. To determine the spectrum, prevalence and predictors of skin-related complications in KTS. A retrospective review of 410 patients fulfilling KTS criteria was performed to assess for the presence of skin-related complications. Skin-related complications were present in 45% of patients. Most prevalent were CM-related complications including blebs, bleeding, thickening (25%), cellulitis (22%) and ulceration (21%). Features positively associated with skin-related complications were presence of LM (OR 17.17; P < 0.001), VM on the buttocks/perineum/genitalia (OR 1.92; P = 0.009), CM on the feet (OR 1.77; P = 0.039) and male sex (OR 1.63; P = 0.034). Features negatively associated with skin-related complications were CM on the trunk (OR 0.59; P = 0.029) and tissue hypertrophy of the hands (OR 0.27; P = 0.025). Skin-related complications affect nearly half of patients with KTS. Those with lymphatic involvement or malformation presence in the undergarment area or feet are most at risk.
Sections du résumé
BACKGROUND
BACKGROUND
Little is known about skin-related complications in Klippel-Trenaunay syndrome (KTS), a complex vascular anomaly defined by capillary malformation (CM), venous malformation (VM) ± lymphatic malformation (LM) and limb overgrowth. Reported skin-related complications of KTS include ulceration, vascular ectasias (blebs), bleeding and infection.
OBJECTIVE
OBJECTIVE
To determine the spectrum, prevalence and predictors of skin-related complications in KTS.
METHODS
METHODS
A retrospective review of 410 patients fulfilling KTS criteria was performed to assess for the presence of skin-related complications.
RESULTS
RESULTS
Skin-related complications were present in 45% of patients. Most prevalent were CM-related complications including blebs, bleeding, thickening (25%), cellulitis (22%) and ulceration (21%). Features positively associated with skin-related complications were presence of LM (OR 17.17; P < 0.001), VM on the buttocks/perineum/genitalia (OR 1.92; P = 0.009), CM on the feet (OR 1.77; P = 0.039) and male sex (OR 1.63; P = 0.034). Features negatively associated with skin-related complications were CM on the trunk (OR 0.59; P = 0.029) and tissue hypertrophy of the hands (OR 0.27; P = 0.025).
CONCLUSION
CONCLUSIONS
Skin-related complications affect nearly half of patients with KTS. Those with lymphatic involvement or malformation presence in the undergarment area or feet are most at risk.
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
517-522Informations de copyright
© 2020 European Academy of Dermatology and Venereology.
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