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Diseases

Genetic and Rare Diseases Information Center (GARD)

Punctate palmoplantar keratoderma type I


Other Names for this Disease
  • Buschke fischer brauer syndrome
  • Keratodermia palmoplantaris papulosa, Buschke-Fischer-Brauer type
  • Type I punctate palmoplantar keratoderma
  • Keratoderma, palmoplantar punctate type 1
  • Keratosis palmoplantaris papulosa
Related Diseases
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Treatment

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How might punctate palmoplantar keratoderma type 1 be treated?

Treatment options for this condition generally include topical salicylic acid, mechanical debridement, excision, and systemic retinoids.[1] These therapies can lead to a temporary decrease in skin thickness and softening of the skin.[2] Unfortunately, in many cases, medical and surgical treatment for punctate keratodermas do not provide consistent or long-lasting results.[3]

In general, there has been some reported success using keratolytics such as corticosteroids, urea, salicylic acid, lactic acid, or Vitamin A. Systemic therapy using vitamin D analogues, aromatic retinoids, and 5-fluorouracil has also been used. However, individuals with successful resolution of lesions often relapse unless they are maintained on chronic low-dose therapy. These topical and systemic treatments carry a variety of side effects.[3]

Surgery (including excision and skin grafting) for punctate keratodermas has been used on lesions resistant to medical treatment, but healing after surgical treatment can be difficult. CO2 laser ablation has also been attempted and reportedly produces good results for limited areas of hyperkeratosis of the palms.[3]
Last updated: 2/1/2016

References
  1. Kingsbery MY. Keratosis Palmaris et Plantaris. Medscape Reference. July 23 2014; http://emedicine.medscape.com/article/1108406-overview#a1. Accessed 12/30/2015.
  2. Oztas P et al.,. Punctate palmoplantar keratoderma (Brauer-Buschke-Fisher Syndrome). Am J Clin Dermatol. 2007;
  3. O'connor EA, Dzwierzynski WW. Palmoplantar keratoderma: treatment with CO2 laser case report and review of the literature. Ann Plast Surg. October 2011; 67(4):439-441.


Clinical Trials & Research for this Disease

  • The Centers for Mendelian Genomics program is working to discover the causes of rare genetic disorders. For more information about applying to the research study, please visit their website.
  • Foundation for Ichthyosis and Related Skin Types has funded ichthyosis-related research through its Research Grant Program.
  • Orphanet lists European clinical trials, research studies, and patient registries enrolling people with this condition. 
Other Names for this Disease
  • Buschke fischer brauer syndrome
  • Keratodermia palmoplantaris papulosa, Buschke-Fischer-Brauer type
  • Type I punctate palmoplantar keratoderma
  • Keratoderma, palmoplantar punctate type 1
  • Keratosis palmoplantaris papulosa
Related Diseases
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.