Skin-Related complications of Klippel-Trenaunay Syndrome: a retrospective review of 410 patients.
J Eur Acad Dermatol Venereol
; 35(2): 517-522, 2021 Feb.
Article
in En
| MEDLINE
| ID: mdl-33070382
ABSTRACT
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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Klippel-Trenaunay-Weber Syndrome
/
Lymphatic Abnormalities
/
Vascular Malformations
Type of study:
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limits:
Humans
/
Male
Language:
En
Journal:
J Eur Acad Dermatol Venereol
Journal subject:
DERMATOLOGIA
/
DOENCAS SEXUALMENTE TRANSMISSIVEIS
Year:
2021
Document type:
Article
Affiliation country: