Olmsted syndrome--congenital palmoplantar and periorificial keratoderma.

Abstract:

:We are reporting the third case of the Olmsted syndrome, an entity that has received little attention in the medical literature. The syndrome consists of congenital diffuse, sharply marginated keratoderma of the palms and soles, onychodystrophy , constrictions of digits, and periorificial keratoses. Our patient, in addition, had congenital universal alopecia, absence of a premolar tooth, leukokeratosis of oral tissue, and hyperlaxity of the joints. The differential diagnosis includes hidrotic ectodermal dysplasia of the Clouston type, pachyonychia congenita, acrodermatitis enteropathica, keratoma hereditarium mutilans of Vohwinkel , mal de Meleda , and other forms of palmoplantar keratodermas . This patient had originally been reported as having acrodermatitis enteropathica, a condition with which the Olmsted syndrome can easily be confused.

journal_name

J Am Acad Dermatol

authors

Poulin Y,Perry HO,Muller SA

doi

10.1016/s0190-9622(84)80264-9

subject

Has Abstract

pub_date

1984-04-01 00:00:00

pages

600-10

issue

4

eissn

0190-9622

issn

1097-6787

pii

S0190-9622(84)80264-9

journal_volume

10

pub_type

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