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. 2011 Sep-Oct;56(5):591-3.
doi: 10.4103/0019-5154.87166.

Olmsted syndrome: report of two cases

G K Tharini  1 , N Hema, S Jayakumar, B Parveen
Affiliations

Olmsted syndrome: report of two cases

G K Tharini et al. Indian J Dermatol. 2011 Sep-Oct.

Abstract

Olmsted syndrome is an uncommon genetic disorder with symmetrical, diffuse, transgredient, mutilating palmoplantar keratoderma and periorificial hyperkeratosis. Olmsted syndrome in a female patient is particularly rare, and we report two unrelated female patients of Olmsted syndrome, who presented with perioral hyperkeratosis and palmoplantar keratoderma. One of our patients also had woolly hair from birth and flexion contracture of a digit, while the other had pseudoainhum. There was no cardiac involvement. Hence, the diagnosis of Olmsted syndrome was made.

Keywords: Olmsted syndrome; palmoplantar keratoderma; perioral hyperkeratosis; woolly hair.

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Conflict of interest statement

Conflict of Interest: Nil.

Figures

Figure 1
Figure 1
Case 1 showing woolly hair, keratoderma of palms and flexion contracture of right little finger
Figure 2
Figure 2
Shows woolly hair, scanty eyebrows and perioral erythema and scaling
Figure 3
Figure 3
Case 2 showing perioral hyperkeratosis
Figure 4
Figure 4
Keratoderma extending over dorsal aspect of hands with keratotic papules and constriction of right little finger
See this image and copyright information in PMC

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