Unsatisfactory response to sirolimus in Maffucci syndrome-associated spindle cell hemangiomas.

Abstract:

:Maffucci syndrome is characterized by multiple benign vascular anomalies and enchondromas present on the distal extremities. Effective treatment options are currently not available for Maffucci syndrome-associated vascular lesions. Sirolimus is a mTOR pathway inhibitor, and has been tried successfully in the treatment of various vascular anomalies. We treated a 23-year-old female with Maffucci syndrome-associated spindle cell hemangiomas with oral sirolimus (2mg/day, 0.04mg/kg/day). There was improvement in pain, but no change in colour or size of the vascular nodules. In view of unsatisfactory response and treatment-related adverse effects (oral aphthae, mild transaminitis), sirolimus was stopped after 6 months.

journal_name

Dermatol Ther

journal_title

Dermatologic therapy

authors

Gupta V,Mridha AR,Khaitan BK

doi

10.1111/dth.12851

subject

Has Abstract

pub_date

2019-05-01 00:00:00

pages

e12851

issue

3

eissn

1396-0296

issn

1529-8019

journal_volume

32

pub_type

信件
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