DOI: http://dx.doi.org/10.18203/issn.2455-4529.IntJResDermatol20172216

A rare association of angiokeratoma circumscriptum with Klippel Trenaunay syndrome

Aarti S. Salunke, Ravindranath B. Chavan, Vasudha A. Belgaumkar, Pallavi P. Patil

Abstract


Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder of blood and lymphatic vessels that is characterised by a combined vascular malformation of the capillaries, veins and lymphatics, congenital abnormalities, and associated limb hypertrophy. It may also involve gastrointestinal tract which can lead to life threatening bleeding. This syndrome presents since birth to early infancy or childhood with equal distribution in both genders. An adolescent female presented with right lower limb hypertrophy with port wine stain and overlying hemorrhagic vesiclessince birth. Histopathology of these hemorrhagic vesicles angiokeratoma. On the basis of history and classical clinical triad patient was diagnosed as KTS with angiokeratoma, an association rarely reported in literature.


Keywords


Klippel Trenaunay syndrome, port wine stain, limb hypertrophy

Full Text:

PDF

References


Kumar KJ, Chavan A, Jain M, Manjunath VG, Satya P. Klippel Trenaunay syndrome. Sri Lanka J Child Health. 2012;41(4):209–10.

Das D, Patil P, Tambe SA, Nayak CS. Angiokeratoma circumscriptum in a child of Klippel-Trenaunay syndrome: A rare association. Indian J Paediatr Dermatol. 2015;16:165-7.

Luks VL, Kamitaki N, Vivero MP, Uller W, Rab R, Bovée JVMG, et al. Lymphatic and other vascular malformative overgrowth disorders are caused by somatic mutations in PIK3CA. J Pediatr. 2015;166(4):1048–54.

Husmann DA, Rathburn SR, Driscoll DJ. Klippel-Trenaunay Syndrome: Incidence and Treatment of Genitourinary Sequelae. J Urol. 2007;177:1244–9.

Oduber CE1, van der Horst CM, Hennekam RC. Klippel-Trenaunay syndrome: diagnostic criteria and hypothesis on etiology. Ann Plast Surg. 2008;60(2):217–23.

Jindal SR, Chalvade P, Jerajani HR. Late onset palmar angiokeratoma circumscriptum: An unusual presentation. Indian Dermatol Online J. 2014;5:320–2.

Gloviczki P, Driscoll DJ. Klippel-Trenaunay syndrome: current management. Phlebol. 2007;22(6):291–8.