Cor Vasa 2010, 52(11-12):729-733 | DOI: 10.33678/cor.2010.180

Vascular skin lesions in a child with Klippel-Trénaunay syndrome

Vladimír Bartoš^1,*, Katarína Adamicová², Milada Kullová³, Oľga Zacharová¹, Tatiana Bulejčíková³, Martin Péč⁴

¹ Oddelenie patologickej anatómie, Fakultná nemocnica s poliklinikou v Žiline

² Ústav patologickej anatómie, Univerzitná nemocnica Martin a Jesseniova lekárska fakulta Univerzity Komenského v Martine

³ Oddelenie dermatovenerológie, Fakultná nemocnica s poliklinikou v Žiline

⁴ Ústav lekárskej biológie, Jesseniova lekárska fakulta Univerzity Komenského v Martine, Slovenská republika

Klippel-Trénaunay syndrome is a rare congenital vascular disorder in which one of the main diagnostic features are capillary skin malformations characterized by naevus flammeus present since birth. However, the clinical symptomatology of the disease is relatively variable with frequent incomplete forms whereby the most important diagnostic feature is its cutaneous manifestation. Beside the capillary anomalies there can be also present multiple hemangiomas, lymphangiomas, angiokeratomas with exceptionally reported malignant skin tumors. The authors present the case report of a 12-year-old boy with Klippel-Trénaunay syndrome focusing on dermatologic disease manifestation with biopsy-based diagnosis of benign vascular tumors of the skin. In the discussion section, the authors address the issue of histomorphologic examination of tumorous and non-tumorous vascular skin lesions in childhood, pitfalls of the differential diagnosis, and highlight the need for close cooperation of the clinician and pathologist in the diagnostic process.

Keywords: Klippel-Trénaunay syndrome; Capillary skin malformations; Hemangiomas

Published: November 1, 2010  Show citation