Page 6 - PowerPoint Presentation

Clinical features

 Patients commonly present with

unilateral upper / lower limb swelling,
sensation of heaviness and possible

cellulitis

 Clinical examination will
demonstrate Kaposi-Stemmer sign

(which is the inability to pinch the
dorsal skin over the base of toe). This

is to rule out lipidemia

Vignes S . Orphanet J Rare Dis. 2021 Jan6;16(1) : 18.