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Case Reports
. 2019 Jul-Aug;10(4):447-452.
doi: 10.4103/idoj.IDOJ_418_18.

Cloves Syndrome: A Rare Disorder of Overgrowth with Unusual Features - An Uncommon Phenotype?

Vikram K Mahajan  1 , Mrinal Gupta  1 , Pushpinder Chauhan  1 , Karaninder S Mehta  1
Affiliations
Case Reports

Cloves Syndrome: A Rare Disorder of Overgrowth with Unusual Features - An Uncommon Phenotype?

Vikram K Mahajan et al. Indian Dermatol Online J. 2019 Jul-Aug.

Abstract

CLOVES syndrome characterized by Congenital Lipomatous Overgrowth, Vascular malformations, Epidermal nevi, and Skeletal anomalies is a recently described sporadic syndrome from postzygotic activating mutations in PIK3CA. This 3-year-old boy, born to nonconsanguineous and healthy parents, had epidermal verrucous nevus, lower limb length discrepancy and bilateral genuvalgum, anterior abdominal wall lipomatous mass, central beaking of L2 and L3, and fibrous dysplasia of the left frontal bone. Ocular and dental abnormalities (ptosis, esotropia, delayed canine eruption, dental hypoplasia), ipsilateral asymmetrical deformity of skull, and large left cerebral hemisphere with mild ipsilateral ventriculomegaly were peculiar to him denoting an uncommon phenotype. The parents did not consent for magnetic resonance imaging and genetic studies because of financial constraints. The CLOVES syndrome has emerged as an uncommon yet distinct clinical entity with some phenotypic variations. Its diagnosis is usually from cutaneous, truncal, spinal, and foot anomalies in clinical and radioimaging studies. Proteus syndrome remains the major differential.

Keywords: Congenital asymmetric overgrowth; PIK3CA-related overgrowth spectrum; Proteus syndrome; congenital lipomatous hamartoma; lipoma; lymphovascular malformation.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
(a) Verrucous epidermal nevus over left side of neck. (b) Verrucous epidermal nevus extending over left axilla and arm. Similar lesions were also present over dorsum of left hand and anterior left thigh. (c) Left ptosis and asymmetric skull. (d) Lipomatous mass of anterior abdominal wall. No overlying phlebectasia is seen
Figure 2
Figure 2
(a) Anterior abdominal wall lipomatous mass in computed tomography abdomen. (b) Fibrous dysplasia (?) of left frontal bone in computed tomography head. (c) Large cerebral volume of left cerebral hemisphere in computed tomography brain. (d) Left ventriculomegaly in computed tomography brain
See this image and copyright information in PMC

References

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