Olmsted syndrome[1,2,3] is a rare form of palmoplantar keratoderma characterized by mutilating transgradient keratoderma and periorificial hyperkeratotic plaques. Most cases are sporadic, with defective expression of keratin 1 and keratin 10 in the involved epidermis. The keratoderma is well defined, erythematous, and associated with fissuring and flexion deformity of the digits, which may lead to spontaneous amputation. The condition is slowly progressive with risk of malignancy. Ocular manifestations include hyperkeratosis of lid margins, corneal dystrophy and opacity, and lacrimal gland involve ment. We report this rare disorder in an 18-month-old girl who had skin and eyelid involvement [Fig. 1]. She was advised oral isotretinoin and topical keratolytic agents.
Figure 1.
Hyperkeratotic lesions (a) on eyelid margins, (b) periorally and nasally, (c) on palms, and (d) on soles
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References
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