Original articlePapular epidermal nevus with “skyline” basal cell layer (PENS)
Section snippets
Patients
Five infants, 2 boys and 3 girls, with similar isolated or multiple skin lesions present since birth were observed over a period of 2 years. Their records and photographs were collected, and clinical data including a description of the skin lesions, their number and distribution, and a complete clinical examination were reviewed. A skin specimen biopsy was obtained from one lesion in every patient, and the samples were fixed in formalin, embedded in paraffin, and stained with hematoxylin-eosin.
Genetic testing
Clinical features
The number of lesions ranged from 1 to 11 and they were widely distributed (Table I). They remained unchanged from birth. No further lesions appeared during a follow-up period ranging from 6 months to 2 years, with the exception of one patient (case 5) who developed a short, linear, hyperkeratotic lesion on her right arm before 1 year of age; a biopsy of this lesion was not permitted by her parents.
The individual nevi consisted of 1- to 7-mm hyperkeratotic papules with a rough, flat surface (
Discussion
This presentation of small, scattered, hyperkeratotic papules with the histopathological features of a keratinocytic nevus and a striking palisading skyline pattern in the basal layer appears to be a distinct congenital skin condition. The lesions did not follow the lines of Blaschko, but were rather round, oval, comma shaped, or polygonal. They did not show a regional predisposition and were found in many different areas of the body. The palms and soles were spared.
Two other conditions were
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2022, Annales de Dermatologie et de VenereologieCitation Excerpt :EN is pink to brown in color and linked to a FGFR3, RAS, PIK3CA mutation [19]. The semiology of these lesions was evocative of Papular Epidermal Naevus with Skyline basal cell layer (PENS), but the histology did not demonstrate the characteristic skyline (Fig. 2) [20]. A similar case of multiple white EN has been reported elsewhere in a child with a TSC2 mutation [21].
Epidermal Nevi and Related Syndromes –– Part 1: Keratinocytic Nevi
2018, Actas Dermo-SifiliograficasPENS (papular epidermal nevus with "skyline" basal cell layer)
2015, Annales de Dermatologie et de VenereologieWhen in doubt look up to the skies: A skywatcher's perspective in dermatology
2023, Indian Journal of Dermatology, Venereology and LeprologyWhite hyperkeratotic plaque in a patient with infantile spasms
2022, Pediatric Dermatology
Funding sources: None.
Conflicts of interest: None declared.