Olmsted Syndrome: Report of Two New Cases and Literature Review

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...

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Bibliographic Details
Published in:Journal of dermatology 2000-09, Vol.27 (9), p.557-568
Main Authors: Larrègue, Marc, Callot, Valérie, Kanitakis, Jean, Suau, A. Montis, Foret, Murielle
Format: Article
Language:English
Subjects:
Child
Contracture - etiology
Diagnosis, Differential
Disease Progression
Facial Dermatoses - congenital
Facial Dermatoses - genetics
Facial Dermatoses - pathology
Humans
keratinization disorders
Keratoderma, Palmoplantar - congenital
Keratoderma, Palmoplantar - genetics
Keratoderma, Palmoplantar - pathology
Male
Neck
Olmsted syndrome
palmoplantar keratoderma
periorificial erythrokeratoderma
Skin Transplantation
Syndrome
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Summary: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.
ISSN:0385-2407
1346-8138
DOI:10.1111/j.1346-8138.2000.tb02229.x