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Olmsted Syndrome: Report of Two New Cases and Literature Review
Author(s) -
Larrègue Marc,
Callot Valérie,
Kanitakis Jean,
Suau A. Montis,
Foret Murielle
Publication year - 2000
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/j.1346-8138.2000.tb02229.x
Subject(s) - history , medicine
Olmsted syndrome is a rare keratinization disorder; 18 cases have been published so far. It associates a mutilating congenital palmoplantar keratoderma with periorificial erythematokeratotic lesions. We report herein two new unrelated male children with Olmsted syndrome (OS), one of whom was studied by light and electronmicroscopy. Our histological, immunohistochemical, and ultrastructural findings suggest that this disease is related to epidermal hyperproliferation. We present herein a review of the twenty cases published so far and discuss the major clinicopathological and genetic features of this disease.