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Papular epidermal nevus with “skyline” basal cell layer (PENS) is a very rare type of keratinocytic nevus and is associated with extracutaneous findings such as neurological symptoms in about 50% of the cases. Therefore, it is also referred to as PENS syndrome. Clinically visible hyperkeratotic papules and plaques already appear at birth or shortly...
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... with focally subcorneal abscesses of neutrophils. Additional investiga- tion employing the marker S-100 did not show any pathological issue, although some S-100 melanocytes with perinuclear halo formation up to the middle of the epidermis were visible. The adjacent dermis is not affected, and therefore no deeper infiltration seems to occur [1] (Fig. ...
Citations
Papular epidermal nevus with “skyline” basal cell layer (PENS) is a type of epidermal nevus presenting as small, flat-topped, hyperkeratotic papules. They may be located anywhere on the skin surface, and can be single or multiple. Histopathology shows a characteristic palisading of the basal cell layer with the “skyline” sign. PENS may present with extracutaneous defects, constituting the so-called PENS syndrome.
Papular epidermal nevus with "skyline" basal cell layer (PENS) is a keratinocytic nevus that can occur sporadically or has a familial transmission. There are 5 families reported with PENS, in which there are 2 family members affected with each case. We present the sixth familial case, with the peculiarity of being the first time in which there are 3 family members with PENS, while reviewing the other cases described until now. In addition, we present a new histopathological finding, an inflammatory lichenoid infiltrate on the upper dermis in PENS lesions. This finding could be the result of trauma to the biopsied lesion, or it may represent a new inflammatory histological variant.
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.
