2020Indian Dermatology Online JournalOpen access

Klippel–Trenaunay syndrome with arterio-veno-lymphatic malformation: A rare presentation

VaishaliH Wankhade, BhagyashreeB Supekar, Apoorva D. Chopkar, Rajesh Singh, Dharitri Bhat, P. Suresh

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Abstract

Klippel-Trenaunay syndrome (KTS) is a rare disorder characterized by triad of vascular malformations, varicose veins, and bony or soft tissue hypertrophy involving an extremity. Port wine stain is the most common vascular malformation associated with this syndrome. Various other rare vascular malformations described are angiokeratomas, angiokeratoma circumscriptum naeviformae, angiodermatitis, etc. This is the first instance, to the best of our knowledge, describing the capillary, venous, and lymphatic malformations in a same case of KTS.

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What this paper is about

Klippel-Trenaunay syndrome (KTS) is a rare disorder characterized by triad of vascular malformations, varicose veins, and bony or soft tissue hypertrophy involving an extremity. Port wine stain is the most common vascular malformation associated with this syndrome. Various other rare vascular malformations described are angiokeratomas, angiokeratoma circumscriptum naeviformae, angiodermatitis, etc. This is the first instance, to the best of our knowledge, describing the capillary, venous, and lymphatic malformations in a same case of KTS.

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Available abstract

Klippel-Trenaunay syndrome (KTS) is a rare disorder characterized by triad of vascular malformations, varicose veins, and bony or soft tissue hypertrophy involving an extremity. Port wine stain is the most common vascular malformation associated with this syndrome. Various other rare vascular malformations described are angiokeratomas, angiokeratoma circumscriptum naeviformae, angiodermatitis, etc. This is the first instance, to the best of our knowledge, describing the capillary, venous, and lymphatic malformations in a same case of KTS.

Key concepts: Klippel-Trenaunay syndrome, Angiokeratoma, Medicine, Port-wine stain, Venous malformation, Vascular malformation, Varicose veins, Lymphatic system

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