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

D. Bessis | Zendy; H. Vernhet | Zendy; M. Bigorre | Zendy; I. Quéré | Zendy; Jochen Rößler | Zendy

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

Author(s) -

D. Bessis,

H. Vernhet,

M. Bigorre,

I. Quéré,

Jochen Rößler

Publication year - 2016

Publication title -

jama dermatology

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 2.128

H-Index - 166

eISSN - 2168-6084

pISSN - 2168-6068

DOI - 10.1001/jamadermatol.2016.1008

Subject(s) - medicine , sirolimus , lymphatic system , venous malformation , thigh , surgery , klippel trenaunay syndrome , dermatology , pathology , soft tissue

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. Prolonged and recurrent cutaneous bleeding from ulceration of the capillary and/or venous and/or lymphatic malformation can be life-threatening. We report for the first time to our knowledge a case of Klippel-Trenaunay syndrome treated by oral sirolimus.

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