Port-wine stain as a clue for two rare coexisting entities

Abstract

Phakomatosis pigmentovascularis (PPV) is an uncommon dermatosis characterised by the presence of both pigmentary and vascular abnormalities. 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.

Concurrent Occurrence of Lobular Capillary Haemangioma and Port-Wine Stain: A Case Report and Literature Review

“…The port-wine stain is a non-neoplastic hamartomatous proliferation of capillaries resulting in nodular growth, as a result of ectatic blood vessels [2,[9][10][11][12]. A port-wine stain clinically presents from birth and such lesions do not regress or undergo involution over a period of time.…”