Port-wine stain as a clue for two rare coexisting entities. (13th July 2018)

• Record Type: Journal Article
• Authors: Almeida, Filipa Tavares; Caldas, Regina; Duarte, Maria da Luz; Brito, Celeste
• Is Part Of: BMJ case reports. Volume 2018
• Publication Date: 2018-07-13
• Subjects: dermatology -- ophthalmology; Medicine -- Case studies -- Periodicals; 610.5
• DOI: 10.1136/bcr-2018-225721
• Languages: English
• ISSNs: 1757-790X

Abstract

Abstract : Phakomatosis pigmentovascularis (PPV) is an uncommon dermatosis characterised by the presence of both pigmentary and vascular abnormalities. 1 Its pathogenesis is not elucidated, and the prognosis is mainly determined by the presence of extracutaneous manifestations, such as Klippel-Trenaunay syndrome (KTS), that is defined by the triad of a port-wine stain (PWS), anomalous veins and progressive overgrowth of the affected extremity. Herein, we report a case of an adult patient, who presented with a large PWS, nevus of Ota, ocular melanosis, and limb hypertrophy and varicosities. These findings represented a rare association of PPV type IIb and KTS.