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Keratosis palmoplantaris papulosa

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


Keratosis palmoplantaris papulosa (KPPP) is a rare condition that affects the skin. It is a type of punctate palmoplantar keratoderma.[1][2][3] Signs and symptoms begin in early adolescence or later and include hard, round bumps of thickened skin on the palms of the hands and soles of the feet.[1][2][4][3][5] KPPP is usually inherited in an autosomal dominant manner and can be caused by mutations in the AAGAB gene.[5] Treatment options may include chemical or mechanical keratolysis as well as systemic acitretin. Some affected individuals have used surgical approaches consisting of excision and skin grafting.[6]


References

  1. Martinez-Mir A et al. Identification of a locus for type I punctuate palmoplantar keratoderma on chromosome 15q22-q24. J Med Genet. 2003.
  2. Oztas P et al.,. Punctate palmoplantar keratoderma (Brauer-Buschke-Fisher Syndrome). Am J Clin Dermatol. 2007.
  3. Asadi AK. Type I hereditary punctuate keratoderma. Dermatology Online Journal. 2003.
  4. Erkek E et al.,. Type I Hereditary punctuate keratoderma associated with widespread lentigo simplex and successfully treated with low-dose oral acitretin. Arch Dermatology. 2006 Aug.
  5. KERATODERMA, PALMOPLANTAR, PUNCTATE TYPE I; PPKP1. OMIM. http://omim.org/entry/148600. Accessed November 9, 2012.
  6. Rapprich S, Hagedorn M. Surgical treatment of severe palmoplantar keratoderma. J Dtsch Dermatol Ges. March 2011.
  7. Palmoplantar keratoderma. DermNet NZ. http://www.dermnetnz.org/scaly/palmoplantar-keratoderma.html. Accessed March 10, 2010.
  8. Punctate-type palmoplantar keratoderma. DermNet NZ. http://www.dermnetnz.org/scaly/punctate-keratoderma.html. Accessed March 10, 2010.
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General Information

  • MedlinePlus provides more information on skin conditions in general. MedlinePlus is a Web site designed by the National Library of Medicine to help you research your health questions.
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  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.  Click on the link to read information on this topic.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Keratosis palmoplantaris papulosa. Click on the link to view a sample search on this topic.
  • The The Online Mendelian Inheritance in Man (OMIM) database contains genetics resources that discuss Keratosis palmoplantaris papulosa. Click on the link to go to OMIM and review these resources.