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Complex Total Hip Replacement in a Patient with Epidermolytic HyperkeratosisA Case Report
Thomas L. Bernasek, MD1; Elizabeth A. Rommel, BA1
1 Florida Orthopaedic Institute, 13020 Telecom Parkway North, Tampa, FL 33637. E-mail address for T.L. Bernasek: tbernasek@floridaortho.com
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The authors did not receive grants or outside funding in support of their research or preparation of this manuscript. They did not receive payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity. A commercial entity (DePuy Orthopaedics) paid or directed, or agreed to pay or direct, benefits to a research fund, foundation, educational institution, or other charitable or nonprofit organization with which the authors are affiliated or associated.
Investigation performed at the Florida Orthopaedic Institute, Tampa, Florida

The Journal of Bone and Joint Surgery, Incorporated
J Bone Joint Surg Am, 2005 Dec 01;87(12):2775-2779. doi: 10.2106/JBJS.D.02415
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Epidermolytic hyperkeratosis is an autosomal dominant ichthyosis caused by a mutation in the genes for keratin-1 (KRT1) or keratin-10 (KRT10)1,2. The prevalence of this disorder has been estimated to be one in 300,000 individuals3,4. The disease is characterized by erythema, scaling (particularly around large joints), and blistering of the skin following trauma3-8. The skin is more susceptible to infection, particularly in areas of hyperkeratosis7,8. A patient with epidermolytic hyperkeratosis may be at a higher risk for infection when undergoing total joint replacement. We are not aware of any previous reports on patients with this condition who were managed with total hip replacement. We describe the case of one such patient and describe the steps that were taken to avoid infection. The patient was informed that data concerning the case would be submitted for publication.
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