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Epidermal hamartoma presenting as longitudinal pachyleukonychia: a new nail genodermatosis
Background:
Epidermal hamartomas involving the nail apparatus are uncommon.
Objective:
Our purpose was to define the clinical, genetic, and histologic features of a unique nail abnormality.
Methods:
We examined eight members of three generations who presented with an unusual nail abnormality, longitudinal pachyleukonychia, as a sole characteristic that appeared between the ages of 9 and 30 years. One nail was examined histologically.
Results:
Histologic examination showed pronounced hyperplasia of the nail bed with papillomatosis. Cell nests of a sebaceous type were included in the epithelial digitations. This dysplasia was not accompanied by abnormalities affecting skin, hair, teeth, or sweat glands.
Conclusion:
This unique epidermal hamartoma appears to be inherited as an autosomal dominant condition.