Klippel-trenaunay syndrome associated with pseudo - Kaposi's sarcoma

NT Irene, OH Hema, V Rajagopalan, B Parveen

 Department of Dermatology, Government Royapettah Hospital, Kilpauk Medical College, Chennai - 600 014, Tamil Nadu, India

Correspondence Address:
B Parveen
Department of Dermatology, Government Royapettah Hospital, Kilpauk Medical College, Chennai - 600 014, Tamil Nadu
India

Introduction

Klippel Trenaunay syndrome is a rare congenital nevoid disorder characterized by vascular nevus formation, venous varicosities and hypertrophy of the affected tissues. Pseudo Kaposi′s sarcoma is an unusual complication associated with vascular malformation occurring with arterio venous fistulae. Very occasionally it has been associated with Klippel Trenaunay sydrome, where the malformation is predominantly venous with arterio venous fistulae.[1] We report here a cese of Klippel Trenaunay syndrome with pseudo Kaposi′s sarcoma.

Case Report

An 18 - year-old boy presented with unilateral limb hypertrophy of the right leg from birth associated with an overlying erythematous patch. He also complained of hyperpigmented swellings over the affected leg of three years duration which had recently ulcerated. Except for prolonged bleeding after trauma, he was largely asymptomatic and did not have any symptoms suggestive of systemic involvement. There was no contributory family history.

On examination, the affected leg was greatly enlarged in size compared to the contralateral leg with an increase in length of about 7 centimeters and an increase in girth of about 9 centimeters. There was a large port wine stain on the lateral aspect of the affected leg extending from the gluteal region right upto the ankle [Figure - 1].Varicosities were present on the medial side extending upto the sapheno femoral junction. There was no bruit, and Bran- hams sign was negative. Three circumscribed swellings were present over the affected leg on the shin and over the dorsum of the foot. They were pigmen- ted,had a crus- fed surface and an ulcerated summit, with a surrounding area of hypertrichosis. Examination of other systems was normal, except for scoliosis.

Routine investigations on blood and urine were within normal limits. Skiagram of the affected leg showed cortical thickening of the long bones. Colour Doppler and Duplex study of the vascular system of the affected leg showed dilated common femoral artery, segmental thrombosis and dilatation of the saphenous vein and the absence of any arterio venous fistulae. Ultrasound of the abdomen was normal.

Histopathology of the pigmented ulcerated swellings showed capillary formation and extravasation of red cells and hemosiderin deposition consistent with pseudo Kaposi′s sarcoma.

Discussion

Klippel Trenaunay syndrome as originally described comprised the triad of portwine stain extending the full length of a limb with venous varicosities of the same limb and overgrowth of all the tissues of the affected limb particularly bone.[2] Subsequently Parkes Weber described a similar syndrome with limb hypertrophy but with aretrio venous fistulae which he called "hemangiectatic hypertrophy".[3] Thus unilateral soft tissue hypertrophy with predominantly venous malformation and without an arterio venous fistula are designated as Klippel Trenaunay sydrome and those with arterio venous fistulae as the disorder described by Parkes Weber. Most authors agree that the two are separate entities.[4] With the characteristic clinical triad and the absence of an arterio venous fistula which was confirmed clinically and by Doppler studies our patient fitted the label of Klippel Trenaunay sydrome.

Pseudo Kaposi′s sarcoma is characterized by violaceous nodules or plaques, usually associated with arterio venous fistulae.[5] Very occasionally they are associated with Klippel Trenaunay syndrome per se.[1] Pseudo Kaposi′s sarcoma has been classified into two types- Stewart Blufarb type associated with arterio venous fistulae, and the Mail type associated with stasis dermatitis. The former occurs early in life and is unilateral whereas the latter occurs late in life and is bilateral.[5]

Our patient was typical case of Klippel Trenaunay syndrome with the associated complications of compensatory scoliosis due to limb lengthening and pseudo Kaposi′s sarcoma. The association of pseudo kaposi′s sarcoma of the Stewart Blufarb type was interesting as it is a reportedly rare complication of Klippel Trenaunay sydrome.

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