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.


CONCLUSIONS
This unique epidermal hamartoma appears to be inherited as an autosomal dominant condition.
Dermatology Department, Hôpital de l'Antiquaille, Lyon.
G
G
Moulin
R
R
Baran
C
C
Perrin
D000328:Adult; D002648:Child; D005260:Female; D006222:Hamartoma / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008297:Male; D008875:Middle Aged; D009260:Nail Diseases / Q000209:etiology* / Q000235:genetics* / Q000473:pathology; D010375:Pedigree; D012871:Skin Diseases / Q000150:complications* / Q000175:diagnosis