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Author: Tae Yeb (Terry) Kim, House Officer, North Shore Hospital, Auckland, New Zealand. DermNet NZ Editor in Chief: Adjunct A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell. April 2020.
CLOVES syndrome is a rare, progressive congenital disorder that involves multiple organs including the skin, the vascular system, and the musculoskeletal system .
CLOVES is a mnemonic acronym for:
CLOVES syndrome is extremely rare — about 40 cases have been reported as of 2020 — and there is no accurate estimation of the incidence or prevalence globally. The mutation is sporadic and there is no known familial predisposition.
CLOVES syndrome is caused by a somatic mosaic gain-of-function mutation (a mutation that occurs after the creation of the zygote) in the PIK3CA gene . It is a part of the PIK3CA-Related Overgrowth Spectrum (PROS), a group of rare overgrowth disorders .
CLOVES syndrome presents at birth with vascular malformations, adipose tissue overgrowth (lipomas), epidermal naevi, and varying degrees of bony deformities . Asymmetrical, disproportionate growth may be evident in prenatal scans .
The characteristic features of CLOVES syndrome include:
The complications of CLOVES syndrome depend on its extent. These can include:
The diagnosis of CLOVES syndrome is based upon:
Radiological findings can support the diagnosis. Vascular malformations and lipomatous masses can be assessed by magnetic resonance imaging (MRI) .
The main disorder to be distinguished from CLOVES is proteus syndrome, in which there is no body overgrowth at birth, and no truncal lipomatous mass, acral, or skeletal abnormalities .
Other differential diagnoses for CLOVES syndrome include:
There is no cure for CLOVES syndrome. Improvements in patient condition have been reported from experimental treatments with:
The prognosis of CLOVES syndrome is usually poor, due to its complications. However, there are indications that treatments with PIK3CA inhibitors will prove effective.
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