A Case of Klippel-Trenaunay Syndrome with Lymphangioma Circumscriptum / 대한피부과학회지

ABSTRACT

Klippel-Trenaunay Syndrome(KTS) is a rare clinical syndrome of children and young adults characterized by port-wine stain, varicose veins and venous malformations, and hypertrophy of soft tissue and bone of the extremity which are involved usually unilateral but occasionally bilateral. Additional variants and complicating features include varicose pulmonary veins, cutaneous lymphangiomas, hemihypertrophy of the face, hyperhidrosis, hypertrichosis, cellulitis, paresthesias, syndactyly, macrodactyly, spina bifida, and decalcification of involved bone. We report a 14-year-old girl with port-wine stain on her both flank and left thigh associated with hypertrophy of left lower extremity. She also has lymphangioma circumscriptum on left thigh, macrodactyly, and syndactyly . To the best of our knowledge, this is the first reported case of KTS with lymphagioma circumscriptum in Korea.