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Multiple proliferating pilar tumors with porokeratotic adnexal ostial nevus: A rare association

Jalal Maghfour, MS, Jennifer Kane, MD, Christopher DiMarco, MD and 2 more

JAAD Case Reports · 2020

doi:10.1016/j.jdcr.2019.12.019 (opens in a new tab)

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Abstract

Porokeratotic adnexal ostial nevus (PAON) is a rare nonhereditary adnexal hamartoma of eccrine and hair follicle components, encompassing porokeratotic eccrine ostial and dermal duct nevus and porokeratotic eccrine and hair follicle nevus. Although PAON has been associated with other syndromes such as palmoplantar syndrome, it has been most commonly associated with a mosaic form of keratitis-ichthyosis deafness syndrome. This is due to mutations in the GJB2 gene that encodes a gap junctional protein, connexin 26, which plays a role in keratinocyte growth and differentiation.

Abstract licence: CC BY-NC-ND

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