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October 26, 2016

Efficacy of Topical Sirolimus (Rapamycin) for the Treatment of Microcystic Lymphatic Malformations

Author Affiliations
  • 1Department of Dermatology, Vascular Anomalies Center, University Clinic of Navarra, Pamplona, Spain
JAMA Dermatol. Published online October 26, 2016. doi:10.1001/jamadermatol.2016.3697

Lymphatic malformations (LMs) are benign vascular lesions that arise from embryological disorders during the development of the lymphatic system and cover a wide spectrum of abnormalities. There are 3 morphologic types of cystic LMs: macrocystic, microcystic, and combined. Microcystic LMs present as clear tiny vesicles that permeate the subcutaneous tissue and muscles. Lymphatic malformations (especially superficial lesions) are usually diagnosed by history and physical examination, although deep or large LMs must be evaluated by magnetic resonance imaging (MRI) to confirm the diagnosis and the extension of the vascular abnormality. In the genitalia, LMs can present as discrete, palpable masses or infiltrative lesions involving several anatomical locations. Microcystic LMs are particularly problematic because they are progressive and tend to grow over time and might recur after treatment. Their 2 most frequent complications are bleeding and infection. We report the case of a young man with microcystic LMs successfully treated with topical sirolimus (also known as rapamycin).

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