Extract
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.