Open AccessPhakomatosis pigmentovascularis presenting with Sturge-Weber syndrome and Klippel-Trenaunay syndrome
Author(s) -
Sumit Sen,
Sanchaita Bala,
Chinmay Halder,
Rahul Ahar,
Anusree Gangopadhyay
Publication year - 2015
Publication title -
indian journal of dermatology/indian journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.395
H-Index - 36
eISSN - 1998-3611
pISSN - 0019-5154
DOI - 10.4103/0019-5154.147801
Subject(s) - sturge–weber syndrome , port wine stain , klippel trenaunay syndrome , medicine , dermatology , nevus of ota , nevus , pathology , soft tissue , laser , physics , optics , melanoma , cancer research
Phakomatosis pigmentovascularis (PPV) is a rare cutaneous disorder characterized by combination of capillary malformation and other pigmented naevi. Four types and two subtypes have been described where subtype 'a' present only with cutaneous form and subtype 'b' also with systemic association like in Sturge-Weber syndrome or Klippel-Trenaunay syndrome. Hereby, we report a case where our patient presented with port-wine stain, Nevus of Ota, Sturge-Weber syndrome, and Klippel-Trenaunay syndrome; which has made it a rare combination.