KID syndrome associated with features of ichthyosis hystrix
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Published on: February 20, 2015
H C Nousari1, A Kimyai-Asadi, J L Pinto
1Department of Dermatology, Johns Hopkins Medical Institutions, Baltimore, Maryland 21205, USA. hnousari@welchlink.welch.jhu.edu
Abstract:
Keratitis-ichthyosis-deafness (KID) syndrome is a congenital ectodermal disorder causing erythrokeratoderma, vascularizing keratitis, and neurosensory deafness. Ichthyosis hystrix is a rare cutaneous disease characterized by well-demarcated, spiky, verrucous, linear plaques that is believed to be a clinical and pathologic chimera of two autosomal dominant diseases: epidermal nevus and epidermolytic hyperkeratosis. We present a patient with the classic triad of KID syndrome with clinical and histologic features of ichthyosis hystrix. This case demonstrates that KID syndrome comprises a spectrum of ectodermal disorders which may include diseases such as hystrix ichthyosis and deafness (HID) syndrome.
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