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- W2000611145 abstract "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.Fig. 1 Photograph of the right hip, showing ichthyotic changes two months before total hip replacement.Aseventeen-year-old girl was referred to our office with a diagnosis of developmental dysplasia of the hips and a history of epidermolytic hyperkeratosis. The patient had been diagnosed with epidermolytic hyperkeratosis at birth but had no family history of the condition. She had been required to take cephalexin daily as a prophylactic measure until the age of ten years, after which it was only prescribed for major skin infections. Daily maintenance of the skin involved soaking in a bath and using lotion or petroleum jelly to hydrate and protect portions of the skin that were raw or cracking.The patient presented with a chief complaint …" @default.
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- W2000611145 date "2005-12-01" @default.
- W2000611145 modified "2023-09-27" @default.
- W2000611145 title "Complex Total Hip Replacement in a Patient with Epidermolytic Hyperkeratosis" @default.
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- W2000611145 doi "https://doi.org/10.2106/jbjs.d.02415" @default.
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