Skin‐Related complications of Klippel–Trenaunay Syndrome: a retrospective review of 410 patients

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 ulceratio...

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Bibliographic Details
Published in:	Journal of the European Academy of Dermatology and Venereology Vol. 35; no. 2; pp. 517 - 522
Main Authors:	Anderson, K.R., Nguyen, H., Schoch, J.J., Lohse, C.M., Driscoll, D.J., Tollefson, M.M.
Format:	Journal Article
Language:	English
Published:	England 01-02-2021
Subjects:	Capillaries Humans Klippel-Trenaunay-Weber Syndrome - complications Klippel-Trenaunay-Weber Syndrome - epidemiology Lymphatic Abnormalities Male Retrospective Studies Vascular Malformations - complications Vascular Malformations - epidemiology
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Summary:	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 To determine the spectrum, prevalence and predictors of skin‐related complications in KTS. Methods A retrospective review of 410 patients fulfilling KTS criteria was performed to assess for the presence of skin‐related complications. 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 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.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	0926-9959 1468-3083
DOI:	10.1111/jdv.16999