Abstract

Congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE) syndrome is a recently delineated disorder that comprises vascular malformations (typically truncal), dysregulated adipose tissue, scoliosis, enlarged bony structures (typically of the legs) without progression, or distorting bony overgrowth. The name CLOVE was subsequently extended to CLOVES to emphasize the association with scoliosis/skeletal and spinal anomalies and seizures/central nervous system malformations. We herein report a very rare case of CLOVES syndrome with the findings of lipomatous overgrowth in the cheek (facial asymmetry), vascular malformation (hemangiomas), epidermal nevi (large port wine stains), and skeletal abnormalities (widened first interdigital space, dystrophia in the nail of the first digit of the right foot, and bilateral hypertrophy of the first digits of the feet).

Highlights

  • Congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE) syndrome is a recently delineated disorder that comprises vascular malformations, dysregulated adipose tissue, scoliosis, enlarged bony structures without progression, or distorting bony overgrowth [1]

  • There was no consanguinity between the parents. On physical examination he had a common port wine stain partially involving the skin overlying the right arm, sternal region in the neck, right temporooccipital region, right leg, anterior and posterior parts of left leg, and sacral region. He had a systolic murmur with a grade of II/VI, an hemangioma with a size of 2 × 1 cm in his lower lip, hypertrophy on left cheek, widened first interdigital space, dystrophia in the nail of the first digit of the right foot, and bilateral hypertrophy of the first digits of the feet (Figure 1)

  • The diagnosis of CLOVES syndrome was established on the basis of the findings of lipomatous overgrowth in the cheek, vascular malformation, epidermal nevi, and skeletal abnormalities

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Summary

Introduction

Congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE) syndrome is a recently delineated disorder that comprises vascular malformations (typically truncal), dysregulated adipose tissue, scoliosis, enlarged bony structures (typically of the legs) without progression, or distorting bony overgrowth [1]. The name CLOVE was subsequently extended to CLOVES to emphasize the association with scoliosis/skeletal and spinal anomalies and seizures/central nervous system malformations [2]. We report a very rare and most recently defined case of CLOVES syndrome

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