The mystery of a crimson leg: A case of Klippel–Trénaunay–Weber syndrome

An 82-year-old male with a congenital skin condition presented with fever, increasing left lower extremity edema, and erythema. Exam was notable for lymphedema, phlebectasia, scattered impetiginous changes, and extensive verrucous to multinodular hemangiomatosis of the left leg, thigh, inguinal region, buttock, and abdomen (Panel A). Cellulitis in the setting of Klippel–Trénaunay–Weber syndrome was diagnosed and the patient was treated successfully with antibiotics and compression wraps.

Panel A

Klippel–Trénaunay–Weber syndrome is a rare congenital vascular disorder characterized by lateralizing cutaneous vascular nevus malformation, venous/lymphatic malformations, and hypertrophy of osseous and/or soft tissue. While most commonly affecting a single lower extremity, the extent of vascular involvement is highly variable and vascular malformations may involve the deeper muscular, osseous, genitourinary, gastrointestinal, or solid organ vascular structure. Limb hypertrophy occurs, as in this case (Panel A), secondary to underlying bone and soft tissue involvement. In a series of 252 patients with Klippel–Trénaunay–Weber syndrome, 98% of patients had capillary malformations (port-wine stain), 72% venous malformations, and 67% limb hypertrophy. All three features were present in 63% of patients. ¹

Common complications in Klippel–Trénaunay–Weber syndrome include cellulitis, edema, stasis dermatitis, skin ulceration, deep venous thrombosis, thrombophlebitis, and bleeding – dependent upon the site of vascular involvement. ¹ Use of compression garments is indicated for patients with recurrent cellulitis, with concomitant benefit for chronic venous insufficiency and/or lymphedema. Prophylactic antibiotics may be appropriate in patients with recurrent cellulitis. Frequent infections can prompt discussion of surgical debulking, although this remains controversial in light of the potential for damage to venous and lymphatic drainage with secondary worsening of edema. ²

Klippel–Trénaunay–Weber syndrome is an uncommon syndrome, most often affecting a single limb, with a triad of port-wine stain, varicose veins, and bony and soft tissue hypertrophies. Common complications include cellulitis, which often can be treated effectively with antibiotics and conservative medical care.

‘Images in vascular medicine’ is a regular feature of Vascular Medicine. Readers may submit original, unpublished images related to clinical vascular medicine. Submissions may be sent to: Mark A Creager, Editor in Chief, Vascular Medicine, via the web-based submission system at http://mc.manuscriptcentral.com/vascular-medicine