Olmsted syndrome. Case report and identification of a keratin abnormality.
BACKGROUND
Olmsted syndrome is a rare disorder characterized by a mutilating palmoplantar keratoderma and periorificial keratotic plaques. It begins in early childhood and is complicated by the development of painful flexion contractures, constrictions, and autoamputations of the digits. Only 11 cases of Olmsted syndrome have been reported to date. However, no biochemical abnormalities in the skin were reported in any of these cases.


METHODS
We report the 12th case of Olmsted syndrome. In addition, we describe a keratin abnormality found in a skin specimen obtained from our patient. The specimen showed a suprabasilar staining pattern with AE1, an antibody that shows only basilar staining in normal skin.


CONCLUSIONS
We report the 12th case of Olmsted syndrome, review the literature, and describe a keratin abnormality that was found in our patient's skin specimen.
Kress
D W
DW
Department of Dermatology, University of Pittsburgh Medical Center, Pa, USA.
Seraly
M P
MP
Falo
L
L
Kim
B
B
Jegasothy
B V
BV
Cohen
B
B
D002648:Child; D005260:Female; D006801:Humans; D007633:Keratins / Q000032:analysis / Q000096:biosynthesis; D007645:Keratoderma, Palmoplantar / Q000378:metabolism / Q000473:pathology*; D013577:Syndrome
D007633:Keratins