Striate Palmoplantar Keratoderma (Brunauer-Fohs-Siemens Syndrome)
Alsharif S*, Alraddadi A and Alluhaibi R
Dermatology Department, King Abdulaziz
Medical City, Jeddah, Saudi Arabia
A 24-year-old man presented with linear hyperkeratotic plaques on the volar
fingers, which he had since childhood. That was exacerbated by manual labour. No
family member was affected with similar condition. Histopathologic and electron
microscopic results were consistent with striate palmoplantar keratoderma.
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. Keratolytics are the mainstay of therapy. Use of topical or oral retinoids
and surgical debridement has also been described.
Striate; Palmoplantar keratoderma; Brunauer-Fohs-Siemens syndrome