Update on Epidermal Nevi and Associated Syndromes

Abstract

There are several different types of epidermal nevi, including keratinocyte epidermal nevi, nevus sebaceous, nevus comedonicus, and Becker nevus. This article highlights the varied clinical and histologic features of epidermal nevi, discusses recent data on pathogenesis, and provides an update on treatment options. Syndromes associated with epidermal nevi also are described.

A Review on Cutaneous and Musculoskeletal Manifestations of CLOVES Syndrome

“… 1 , 5 , 7 , 10 , 15 , 21 There is complex and combined deposition of vascular tissue in CLOVES syndrome with a blended mixture of capillary (low-flow and geographic), venous (superficial phlebectasia), lymphatic (macrocystic, microcystic or mixed), and arteriovenous malformations with or without arteriovenous fistulae (high-flow). 1 , 7 , 9 , 12 , 14 , 21 , 27 , 36 , 38 , 39 , 41 , 42 The lesions might be solitary or multiple, localized or diffuse, superficial, or deeply located. 41 Capillary and lymphatic malformations are the most common type of vascular malformations.…”

“…15 Epidermal nevus (particularly linear keratinocytic nevus) is a common, albeit not a universal feature, in CLOVES syndrome. 42 , 47 Some port wine stains accompanying CLOVES syndrome have been erroneously reported as epidermal nevi in the pediatrics literature. 40 In contrast to Proteus syndrome, connective tissue nevus (elastoma, collagenoma) is not a component of CLOVES syndrome.…”

“… 5 , 7 , 12 , 21 , 31 , 38 , 39 , 52 , 54 Overgrowth in CLOVES syndrome is detailed as “ballooning” (gradual increase in the volume of the soft tissue mass), congenital, slowly progressive, mild, symmetric, and proportionate, while that in Proteus syndrome is acknowledged as distorted, postnatal, rapidly progressive, severe, asymmetric, and disproportionate (out of proportion to normal body growth). 1 , 10 , 14 , 18 , 31 , 40 , 42 , 54 CLOVES syndrome and KTS may be distinguished by the absence of truncal involvement and preferential affection of the lower extremities in the latter. 14 , 15 , 21 , 46 High-flow AVM and spinal/ paraspinal AVM are features of CLOVES syndrome and Parkes-Weber syndrome, but not that of KTS.…”