title: Phakomatosis pigmentovascularis presenting with sturge-weber syndrome and klippel-trenaunay syndrome.
abstract: 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.
authors: Department of Dermatology Venereology and Leprosy, Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Kolkata, West Bengal, India.
authors: Sumit
authors: S
authors: Sen
authors: Department of Dermatology Venereology and Leprosy, Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Kolkata, West Bengal, India.
authors: Sanchaita
authors: S
authors: Bala
authors: Department of Dermatology Venereology and Leprosy, Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Kolkata, West Bengal, India.
authors: Chinmay
authors: C
authors: Halder
authors: Department of Dermatology Venereology and Leprosy, Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Kolkata, West Bengal, India.
authors: Rahul
authors: R
authors: Ahar
authors: Department of Dermatology Venereology and Leprosy, Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Kolkata, West Bengal, India.
authors: Anusree
authors: A
authors: Gangopadhyay
keywords: Klippel-Trenaunay syndrome; Sturge-Weber syndrome; phakomatosis
references: J Dermatol. 2003 May;30(5):381-8
references: 12773803
references: Hautarzt. 1989 Nov;40(11):721-4
references: 2606671
references: Eur J Dermatol. 1998 Dec;8(8):569-72
references: 9889430
references: Indian J Dermatol Venereol Leprol. 2010 May-Jun;76(3):307
references: 20445318
references: J AAPOS. 2007 Aug;11(4):398-9
references: 17512229
references: Childs Nerv Syst. 1993 Apr;9(2):107-9
references: 8319229