Images In...
Port wine stain, soft tissue bone hypertrophy and leg ulcer
Clinical center Nis, Dermatology and Venerology, Bld. Zorana Djidnjica 48, Nis, 18000, Serbia
Correspondence to:
Aleksandar Jankovic, jakesnage{at}gmail.com
Vascular congenital malformations and the associated consequences commonly present with skin abnormalities including varicosities, leg ulcers and soft tissue hypertrophy.1 This point is illustrated here.
We present the case of a 67-year-old male patient with a 2 year history of recalcitrant leg ulcer overlying an extensive vascular malformation and varicosities. Clinical examination revealed asymmetric hypertrophy of the upper and lower extremities, an extensive geographic port wine stain which severely affected the face, trunk, both legs, arms, as well as prominent venous varicosities of both legs and truncal abdominal veins (fig 1). Upper and lower limb measurements on the right side were larger than those on the left side (fig 2). The chronic wound was diagnosed as ulceration due to venous stasis and lymphoedema (fig 3). Magnetic resonance imaging scans of both legs confirmed the presence of a venous–lymphatic vascular malformation involving the whole subcutis. These features suggest Klippel–Trenauny syndrome (KTS).
 View this figure (78K): Figure 1 Clinical examination revealed asymmetric hypertrophy of the upper and lower extremities, an extensive geographic port wine stain which severely affected the patient’s face, trunk, both legs, arms, as well as prominent venous varicosities of both legs and trunk abdominal vein.
|
 View this figure (101K): Figure 2 Right hands and feet showed striking palmoplantar hypertrophy, and macrodactyly. Right arm is 2 cm longer than left, and right palm size is 4 cm larger than left. Width and length of the right leg are both 1 cm longer than the left, and the right foot is 1 cm longer than the left.
|
 View this figure (112K): Figure 3 The chronic wound was diagnosed as venous stasis ulceration.
|
Evaluation and management of KTS should be individualised and depends on the age of the patient and any additional signs or symptoms.2 Non-surgical treatment in patients with KTS includes compression therapy (such as with elastic support) of the limb (particularly to try to heal leg ulceration), antibiotics when skin infection is present, possibly diuretics for oedema of lower limbs, and anticoagulants if deep vein thrombosis occurs.3
Competing interests: none.
Patient consent: Patient/guardian consent was obtained for publication
- Lisko, JH, & Fish, F. Klippel-Trenaunay-Weber Syndrome. Arch Dermatol 2003; 139: 1494–6.
[Free Full Text] - Noel, A, Gloviczki, P, Cherry, K, et al. Surgical treatment of venous malformations in Klippel-Trénaunay syndrome. J Vasc Surg 2000; 32: 840–7.[CrossRef][Medline]
- Cohen, MM. Klippel-Trenaunay syndrome. Am J Med Genet 2000; 93: 171–5.[CrossRef][Medline]
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
-
Extract
- Submit a response
- Alert me when this article is cited
- Alert me when eLetters are posted
- Alert me if a correction is posted
Services
- Email this link to a friend
- Similar articles in this journal
- Add article to my folders
- Download to citation manager
- Request Permissions
Citing Articles
Google Scholar
PubMed
Topic Collections
Bookmark with
Register for free content
The full text of all Editor's Choice articles and summaries of every article are free without registration
The full text of Images in ... articles are free to registered users
Only fellows can access the full text of case reports (apart from Editor's Choice) - become a fellow today, or encourage your institution to, so that together we can grow and develop this resource
Don't forget to sign up for content alerts so you keep up to date with all the case reports as they are published, and let us know what you think by commenting on the Editor's blog
