Acral Plexiform Palisaded Encapsulated Neuromas as the Initial Cutaneous Manifestation of Cowden Syndrome

Elizabeth Harris, Adnan Mir

Research output: Contribution to journalArticlepeer-review

Abstract

Cowden syndrome (CS) is an autosomal dominant genodermatosis associated with characteristic mucocutaneous findings of facial trichilemmomas, palmoplantar keratoses, sclerotic fibromas, and oral papillomas. Mucocutaneous neuromas have also been reported in association with CS. We describe a patient with CS whose sole cutaneous finding was palisaded encapsulated neuromas (PENs) with a plexiform growth pattern in an acral location. Along with previous reports, this case suggests that acral plexiform PENs may be an early, highly specific finding in CS and highlights the importance of screening these patients for PTEN mutation.

Original languageEnglish (US)
Pages (from-to)e219-e220
JournalPediatric Dermatology
Volume34
Issue number4
DOIs
StatePublished - Jul 1 2017

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Dermatology

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