Familial papular epidermal nevus with “skyline” basal cell layer

Abstract

Papular epidermal nevus with "skyline" basal cell layer is a variant of keratinocytic nevus that usually occurs sporadically but may affect different family members. We report on the fourth family with papular epidermal nevus with "skyline" basal cell layer affecting a 3-month-old girl and her father.

Hypopigmented Papules in Infancy: A Diagnostic Challenge: Answer

“…Compact orthokeratotic hyperkeratosis, acanthosis with broad and rectangular rete ridges, and a striking palisading of the basal cell layer is characteristic. 11 Epidermal melanocytes may be seen with perinuclear halo formation up to the middle of the epidermis, as shown on S100 immunohistochemical staining. 13 Less common features may include an absent stratum granulosum, or subcorneal neutrophilic abscesses in the stratum corneum.…”

“…7 Cutaneous associations such as pilomatricomas have been reported in PENS, 12 whereas malignant transformation of PENS or association with other malignancies has not been reported. [10][11][12] In conclusion, PENS is a rare subtype of epidermal nevi that is not commonly recognized and under diagnosed. Because of its associations with neurological disorders, dermatologists and dermatopathologists need to have a high index of suspicion for early accurate diagnosis and timely interventions.…”

“…The color of lesions can range from white to brown, [4][5][6]10,13 and in some cases, lesions of different color may be present in the same individual. 7,10,11 Unlike epidermal nevi that occur linearly along lines of Blaschko, 4 PENS lesions do not typically follow a similar distribution and mostly occur in a sporadic distribution, with a predilection for the face, neck, trunk, and extremities, while sparing the palms and soles. 4 Only 2 PENS cases have been described following a Blaschkoid distribution.…”

“…further 5 cases having developed PENS lesions within their first year of life. [2][3][4][5][6][7][8][9][10][11][12][13] In 2 cases, the exact age of development had not been reported. 6,10,11 New lesions can develop within months or up to 3 years after onset, and the total number may go up to as high as 18.…”

“…[2][3][4][5][6][7][8][9][10][11][12][13] In 2 cases, the exact age of development had not been reported. 6,10,11 New lesions can develop within months or up to 3 years after onset, and the total number may go up to as high as 18. 3,4,6 Dermoscopic patterns have been identified in PENS, but may not be diagnostic given the variance between each description and the paucity of reported cases.…”