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Annales de Dermatologie et de Vénéréologie
Volume 141, n° 8-9
pages 507-513 (août 2014)
Doi : 10.1016/j.annder.2014.04.119
Received : 9 January 2014 ;  accepted : 2 April 2014
Syndrome CLOVES : un syndrome malformatif proche du syndrome de Protée
CLOVES syndrome: A malformational syndrome closely resembling Proteus syndrome
 

A. Guillet a, , H. Aubert a, M.-H. Tessier a, A. David b, C. Perret c, M. Penhoat a, J.-F. Stalder a, S. Barbarot a
a Service de dermatologie, CHU Hôtel-Dieu, 1, place Alexis-Ricordeau, 44093 Nantes, France 
b Service de génétique médicale, CHU Hôtel-Dieu, 1, place Alexis-Ricordeau, 44093 Nantes, France 
c Service de radiologie, CHU Hôtel-Dieu, 1, place Alexis-Ricordeau, 44093 Nantes, France 

Auteur correspondant.
Résumé
Introduction

Le syndrome CLOVES (Congenital Lipomatous asymmetric Overgrowth of the trunk with lymphatic, capillary, venous, and combined-type Vascular malformations, Epidermal naevi, Skeletal anomalies) est un syndrome malformatif sporadique récemment décrit (mutation de PIK3CA ), associant une hémi-hypertrophie corporelle, des hamartomes lipomateux du tronc et des malformations multiples.

Observation

Nous rapportons un cas de syndrome CLOVES initialement confondu avec un syndrome de Protée, révélé par un épisode de surinfection d’un hamartome lipomateux du dos. La patiente avait une hypertrophie et des malformations capillaires et veino-lymphatiques du membre inférieur droit, des malformations capillaires dorsales, une hémi-hypertrophie cervico-faciale gauche et un hamartome épidermique cervical, compatibles avec un syndrome de Protée. Le diagnostic de syndrome CLOVES était posé plus tard, devant la mise en évidence par l’imagerie de l’hamartome lipomateux du dos associé aux malformations capillaires mais aussi à des malformations veino-lymphatiques sous-jacentes et à une syringomyélie.

Discussion

Plusieurs syndromes malformatifs peuvent être évoqués devant une hypertrophie tissulaire associée à des hamartomes et à des malformations vasculaires. Ainsi, le diagnostic de syndrome CLOVES peut être retardé car ce syndrome est assez proche phénotypiquement du syndrome de Protée. Cependant, le pronostic et les complications sont différents. Il est important d’évoquer le diagnostic de syndrome CLOVES devant l’association d’hamartomes lipomateux du dos, d’une hémi-hypertrophie corporelle et d’un hamartome épidermique, afin de mettre en place un suivi adapté et une surveillance des complications propres à cette maladie.

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Summary
Background

CLOVES syndrome (Congenital Lipomatous asymmetric Overgrowth of the trunk with lymphatic, capillary, venous, and combined-type Vascular malformations, Epidermal nevi, Skeletal anomalies) is a sporadic malformational syndrome that has recently been described (mutation of PIK3CA ), with asymmetric body hypertrophy, lipomatous hamartoma of the trunk and numerous malformations.

Patients and methods

We report a case of CLOVES syndrome initially diagnosed as Proteus syndrome, revealed by infection of a dorsal lipomatous hamartoma. The patient presented with both right lower limb hypertrophy and capillary and venous-lymphatic malformations, associated with dorsal capillary malformations, left cervico-facial hypertrophy, and cervical epidermal hamartoma, all of which are consistent with Proteus syndrome. Imaging of the dorsal lipomatous hamartoma associated with capillary as well as underlying venous-lymphatic malformations and syringomyelia resulted in subsequent correction of the earlier diagnosis of Proteus syndrome to that of CLOVES syndrome.

Discussion

Several malformational syndromes are associated with tissue hypertrophy, hamartoma and vascular malformations. Diagnosis of CLOVES syndrome may be delayed due to its fairly close phenotypic similarity to Proteus syndrome. Nevertheless, the prognosis and complications differ. Our case underlines the importance of considering a diagnosis of CLOVES syndrome in the presence of lipomatous hamartoma with hemi-hypertrophy and epidermal hamartoma, in order to enable adequate follow-up with specific monitoring for the possible complications associated with this disease.

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Mots clés : Syndrome CLOVES, Syndrome de Protée, Hamartome lipomateux, Hémi-hypertrophie corporelle, Syringomyélie

Keywords : CLOVES syndrome, Proteus syndrome, Lipomatous hamartoma, Hemi-hypertrophy, Syringomyelia




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