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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
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Keratosis palmoplantaris papulosa (KPPP) is a rare condition that affects the skin. It is a type of punctate palmoplantar keratoderma. 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. KPPP is usually inherited in an autosomal dominant manner and can be caused by mutations in the AAGAB gene. 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.
- Martinez-Mir A et al. Identification of a locus for type I punctuate palmoplantar keratoderma on chromosome 15q22-q24. J Med Genet. 2003.
- Oztas P et al.,. Punctate palmoplantar keratoderma (Brauer-Buschke-Fisher Syndrome). Am J Clin Dermatol. 2007.
- Asadi AK. Type I hereditary punctuate keratoderma. Dermatology Online Journal. 2003.
- 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.
- KERATODERMA, PALMOPLANTAR, PUNCTATE TYPE I; PPKP1. OMIM. http://omim.org/entry/148600. Accessed November 9, 2012.
- Rapprich S, Hagedorn M. Surgical treatment of severe palmoplantar keratoderma. J Dtsch Dermatol Ges. March 2011.
- Palmoplantar keratoderma. DermNet NZ. http://www.dermnetnz.org/scaly/palmoplantar-keratoderma.html. Accessed March 10, 2010.
- Punctate-type palmoplantar keratoderma. DermNet NZ. http://www.dermnetnz.org/scaly/punctate-keratoderma.html. Accessed March 10, 2010.
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- 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.