Epidermal nevus syndrome

Abstract

Epidermal nevus syndrome is a group of congenital neurocutaneous disorders characterized by epidermal nevi in association with cerebral, ocular, skeletal, and sometimes cardiac and renal abnormalities. These nevi have been classified according to their predominant component. We described a child presenting with inflammatory linear verrucous epidermal nevus on the head, which is an uncommon location. He also encountered ocular and central nervous system structural disorders.

The group of epidermal nevus syndromes

“…Others had, in fact, a noninflammatory EN. 36,37 Vujevich and Mancini 38 described a boy with multiple ocular, cerebral, and skeletal defects, dysplasia of the large vessels, hydronephrosis with hydroureter, and unilateral sensorineural hearing loss. The associated systematized epidermal nevus may or may not have been a sebaceous nevus, which is why this case cannot be categorized with certainty.…”

The group of epidermal nevus syndromes

“…23 It is the best known ENS, and some authors still prefer to categorize it as ''the ENS.'' [24][25][26][27][28][29][30] The terminology of this birth defect is rather confusing and includes, in addition to Schimmelpenning syndrome, 5,21,31 many other names, such as Feurstein, FeuersteinFeurstein, Feuerstein Mims syndrome, [32][33][34] Schimmelpenning-Feuerstein-Mims syndrome, 35-37 Solomon syndrome, [38][39][40] ENS, 12,38,[41][42][43][44][45][46] Jadassohn nevus phacomatosis, [47][48][49] Jadassohn-Schimmelpenning-Feuerstein-Mims syndrome, 50 organoid nevus phacomatosis, 35,51 organoid nevus syndrome, 52,53 sebaceous nevus syndrome, [54][55][56][57][58] linear sebaceous nevus syndrome, 50,[59][60][61][62][63][64] and Jadassohn sebaceous nevus syndrome. 65 Consequently, Schimmelpenning syndrome temporarily had three different Online Mendelian Inheritance in Man numbers; these have resently been reduced to two entries (163200 and 601359).…”

Epidermolytic hyperkeratosis in inflammatory linear verrucous epidermal nevus

“…[5] About 6% of all epidermal nevi are ILVEN. [67] The lesions consisted of pruritic, erythematous, hyperkeratotic, linear plaques, distributed unilaterally with greater involvement of the left side. [4689]…”

“…[712] Only 16% of lesions were found on the upper half of the body, including the axillae, arm, and hand. [7] A case of ILVEN was also reported on the head. [7]…”