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Papular epidermal nevus with "skyline" basal cell layer (PENS).
Antonio Torrelo1, Isabel Colmenero, Leonard Kristal
1Department of Dermatology, Hospital del Niño Jesús, Madrid, Spain. atorrelo@aedv.es
Journal of the American Academy of Dermatology
|February 15, 2011
Summary
A new type of epidermal nevus, termed papular epidermal nevus with 'skyline' basal cell layer (PENS), is described in five infants. This distinct keratinocytic nevus presents with unique clinical and histopathologic features.
Area of Science:
- Dermatology
- Histopathology
- Genetics
Background:
- Epidermal keratinocytic nevi encompass several recognized subtypes.
- Distinctive clinical and histopathologic features of a novel keratinocytic nevus were investigated.
Purpose of the Study:
- To characterize a previously unreported type of keratinocytic nevus in five patients.
- To document the clinical presentation and histopathologic findings of this new condition.
Main Methods:
- Clinical and photographic review of five affected infants.
- Histopathologic examination of skin biopsy specimens.
- Genetic analysis for specific hotspot mutations in FGFR3 and PIK3CA genes.
Main Results:
- Five infants presented with 1-11 congenital hyperkeratotic papules (1-7 mm) of varying shapes.
- Histopathology revealed acanthosis, broad rectangular rete ridges, and basal cells with palisaded nuclei.
- Genetic testing excluded hotspot mutations in FGFR3 and PIK3CA.
Conclusions:
- A novel keratinocytic nevus, papular epidermal nevus with 'skyline' basal cell layer (PENS), is identified.
- This condition is characterized by specific clinical and histopathologic findings.
- Further research is warranted to understand the etiology and prevalence of PENS.
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