Papular epidermal nevus with “skyline” basal cell layer (PENS)

Torrelo, Antonio; Colmenero, Isabel; Kristal, Leonard; Navarro, Lourdes; Hafner, Christian; Hernández‐Martín, Ángela; Requena, Luis; Happle, Rudolf

doi:10.1016/j.jaad.2010.02.054

Cited by 31 publications

(43 citation statements)

References 12 publications

(8 reference statements)

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“…On the contrary, patient 3 needed further treatment with lamotrigine 50 mg twice daily to stop seizures. Patient 5 had no neurologic signs and symptoms and is very similar to the report of Torrelo et al [2]. Patient 6 has so far not been examined by a neurologist.…”

Section: Discussionsupporting

confidence: 82%

“…However, sporadic cases seem to occur more commonly. Apparently, the observed unusual cutaneous lesions are identical with papular epidermal nevi with ‘skyline’ basal cell layers (PENS), as recently described by Torrelo et al [2]. …”

Section: Discussionsupporting

confidence: 73%

“…In 2006, these unusual cases were included in an ‘Atlas of Genodermatoses’ as examples of a new neurocutaneous syndrome [1]. In 2011 Torrelo et al described five unrelated children with ‘papular epidermal nevus with “skyline” basal cell layer’ and proposed the acronym PENS [2]. The lesions they described were very similar, both clinically and histopathologically, to those observed in our cases.…”

Section: Introductionsupporting

confidence: 63%

“…Lesions have constantly a slightly rough and scaly surface, which may become smooth due to climatic conditions or therapy. Histopathological clues are: (1) a sort of ‘ribbon’ or ‘palisade’ (‘skyline’ [2]) of cuboidal-polygonal dark basal keratinocytes with thickened basal membrane; (2) mild acanthosis; (3) slight orthokeratotic hyperkeratosis. The dermis is normal and, in particular, inflammatory infiltrate is absent.…”

Section: Discussionmentioning

confidence: 99%

“…Their patients had 1–11 keratinocytic nevi measuring 1–7 mm in diameter. The surface was rough and the shape was round, comma-like, rectangular or polygonal [2]. None of their patients, however, had neurologic abnormalities.…”

Section: Introductionmentioning

confidence: 99%

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PENS Syndrome: A New Neurocutaneous Phenotype

et al. 2012

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Background: The central nervous system originates ontogenetically from the ectoderm and therefore numerous neurocutaneous syndromes exist. Objective: We describe a novel neurocutaneous syndrome with characteristic epidermal papules and plaques associated with mild psychomotor retardation and epilepsy with a good prognosis. Methods: We investigated history, clinical features, laboratory parameters including karyotype, histopathological and immunohistological examination, electron microscopy, HPV infection, EEG and neurologic imaging in patients with an unusual symptom complex. Results: Six children are reported with characteristic keratotic papules and plaques of ‘gem-like’ appearance, randomly distributed on the trunk and the proximal part of the limbs. Four patients were unrelated and two were siblings. The cutaneous lesions appeared congenitally or developed soon after birth. The skin lesions were rounded or of an irregularly polycyclic shape, ranging from 0.5 to 1.5 cm in diameter. A few to eighteen lesions were present. The lesional surface was rough and of white or yellowish color. Histopathological examination showed acanthokeratosis, but immunostaining for keratins and melanocytes, electron microscopy and laboratory results were within normal range. During the natural course of the disease the lesions were stable and asymptomatic. All but one of our six patients developed neurologic signs and symptoms during the first year of life, in the form of epilepsy and psychomotor delay. Five children had low intellectual performance together with EEG abnormalities and some had MRI changes of the brain. One patient had marked language impairment. The central nervous system involvement had a peculiar benign and self-limiting course and total normalization occurred in one case. Limitations: We present only a small series of patients. Conclusion: Cutaneous lesions showing identical clinical and microscopical features, but without any extracutaneous abnormalities, have recently been described by Torrelo et al. [J Am Acad Dermatol 2011;64:888–892] under the designation ‘papular epidermal nevus with “skyline” basal cell layer (PENS)’. An association of such nevi with neurologic abnormalities had so far only been published in the ‘Atlas of Genodermatoses’ edited by Caputo and Tadini. We believe that this unique and otherwise undescribed symptom complex is a new neurocutaneous phenotype that may be called PENS syndrome. Whether this new syndrome can be categorized as a distinct phenotype within the group of epidermal nevus syndromes remains speculative and has to be clarified by further investigations.

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Section: Discussionsupporting

confidence: 82%

Section: Discussionsupporting

confidence: 73%

Section: Introductionsupporting

confidence: 63%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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PENS Syndrome: A New Neurocutaneous Phenotype

et al. 2012

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Congenital Naevi and Other Developmental Abnormalities Affecting the Skin

Kinsler

Sebire

2016

Rook's Textbook of Dermatology, Ninth Edition

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The understanding of congenital naevi has evolved rapidly in the last few years, with the discovery of many of the causative genes, and an increasing understanding of the phenotypic correlates of genotypic mosaicism in the skin. A new classification of all congenital naevi is proposed, based on phenotype, histology and genetics, which allows for the addition of new conditions as they are discovered, and for the regrouping of previously distinct disorders. The clinical features and extracutaneous associations of different congenital naevi are reviewed, as well as those of non‐naevoid developmental abnormalities of the skin.

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Epidermal Naevi

Groesser¹,

Hafner²

2019

Harper's Textbook of Pediatric Dermatology

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Papular epidermal nevus with “skyline” basal cell layer (PENS)

Cited by 31 publications

References 12 publications

PENS Syndrome: A New Neurocutaneous Phenotype

PENS Syndrome: A New Neurocutaneous Phenotype

Congenital Naevi and Other Developmental Abnormalities Affecting the Skin

Epidermal Naevi

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