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Porokeratotic Eccrine Duct and Hair Follicle Nevus (PEHFN) Associated with Keratitis-Ichthyosis-Deafness (KID) Syndrome

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Porokeratotic eccrine ostial and dermal duct nevus is a rare hamartomatous malformation, histologically characterized by cornoid lamellae overlying dilated eccrine ostia. The nevus most commonly presents in the form of multiple filiform keratotic spines in a linear arrangement, usually on the distal extremities ( 1). Porokeratotic eccrine and hair follicle nevus is thought to be a variant of porokeratotic eccrine ostial and dermal duct nevus that additionally involves hair follicle infundibula. We report a case of widespread Porokeratotic eccrine and hair follicle nevus that developed in a 15-year-old woman with keratitis-ichthyosis-deafness syndrome.
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Document Type: Research Article

Affiliations: 1: Department of Dermatology, Rhode Island Hospital, The Warren Alpert Medical School of Brown University, Providence, Rhode Island 2: Department of Internal Medicine, University of North Carolina, Chapel Hill, North Carolina 3: Department of Dermatology, The Permanente Medical Group, Pleasanton, California

Publication date: September 1, 2010

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