Striate palmoplantar keratoderma (Brunauer-Fohs-Siemens syndrome)

Jennifer Bragg, Carina Rizzo, Stephanie Mengden

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

Abstract

A 64-year-old man presented with focal hyperkeratotic plaques on the fingers, palms, and soles. Histopathologic and electron microscopic results were consistent with striate palmoplantar keratoderma. Treatment with topical keratolytics was unsuccessful. Striate palmoplantar keratoderma or Brunauer-Fohs-Siemens syndrome is an autosomal dominant condition that presents with linear hyperkeratosis on the palms and fingers and focal plaques on the plantar aspects of the feet. Histopathologic features include hyperkeratosis, hypergranulosis, and acanthosis with no epidermolysis. Electron microscopic examination shows diminished desmosomes, clumped keratin filaments, and enlarged keratohyalin granules. The syndrome has been linked to mutations in desmoglein 1, desmoplakin, and keratin 1. Treatment may include keratolytics, oral retinoids, and surgical debridement.

Original languageEnglish (US)
Article number26
JournalDermatology Online Journal
Volume14
Issue number5
StatePublished - May 2008
Externally publishedYes

ASJC Scopus subject areas

  • Dermatology

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