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Life-Threatening Cutaneous Bleeding in Childhood Klippel-Trenaunay Syndrome Treated With Oral Sirolimus

Didier Bessis, MD1,2,3; Hélène Vernhet, MD, PhD4; Michèle Bigorre, MD5; Isabelle Quéré, MD, PhD6; Jochen Rössler, MD7
[+] Author Affiliations
1Department of Dermatology, Saint-Eloi Hospital, University Hospital of Montpellier, Montpellier, France
2University of Montpellier, Montpellier, France
3INSERM U1058, Montpellier, France
4Department of Radiology, Lapeyronie Hospital, University Hospital of Montpellier, Montpellier, France
5Department of Pediatric Infantile Surgery, Lapeyronie Hospital, University Hospital of Montpellier, Montpellier, France
6Department of Internal Medicine, Saint-Eloi Hospital, University Hospital of Montpellier, Montpellier, France
7Department of Pediatric Oncology and Hematology, Arnaud de Villeneuve Hospital, University Hospital of Montpellier, Montpellier, France
JAMA Dermatol. 2016;152(9):1058-1059. doi:10.1001/jamadermatol.2016.1008.
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This case report describes life-threatening cutaneous bleeding in a child with Klippel-Trenaunay syndrome treated with oral sirolimus

Klippel-Trenaunay syndrome (KTS) is characterized by the triad of cutaneous capillary malformations (port-wine stains), asymmetrical disturbed growth of soft tissues and/or bone, and venous and lymphatic malformations.1 Prolonged and recurrent cutaneous bleeding from ulceration of the capillary and/or venous and/or lymphatic malformation can be life-threatening.2 We report for the first time to our knowledge a case of Klippel-Trenaunay syndrome treated by oral sirolimus.

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Figure 1.
Clinical Efficacy of Sirolimus in Treating Lymphatic Malformation of Klippel-Trenaunay Syndrome

A, Hypertrophy of the lower right limb, multiple port-wine stains on the outside surface of the thigh and knee centered by lymphatic malformations 2 to 13 cm long and composed of grouped hemorrhagic lesions, more rarely whitish or translucid, vesicles, and hemorrhagic crusts. B, Significant reduction in the thickness of the lymphatic malformation as well as its hemorrhagic component after 6 months of oral sirolimus treatment.

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Figure 2.
Magnetic Resonance Imaging Study (T2-Weighted Fat-Saturation Axial Images) of Lymphatic Malformation of Klippel-Trenaunay Syndrome

A, Microcystic lymphangiomatous cutaneous (arrows) and muscular infiltration of the right thigh. B, Marked reduction of the cutaneous lymphatic infiltration (arrows) after 6 months of oral sirolimus therapy.

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