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A disease characterized by keratoderma, hypotrichosis, and leukonychia totalis.

Keratoderma Hypotrichosis Leukonychia Syndrome.

One Turkish female and her two daughters have been described.

The family presented with congenital hypotrichosis, dry skin, keratosis pilaris, and leukonychia totalis. Hair characteristics included trichorrhexis nodosa and trichoptilosis (longitudinal splitting of the distal end of the hair). The patients gradually developed palmoplantar keratoderma and hyperkeratotic lesions on their knees, elbows, and perianal area.

The described features of this syndrome were strictly confined to the skin, and no anesthetic implications are expected.

The syndrome must be distinguished from other forms of palmoplantar keratoderma (Keratosis Palmoplantaris with Esophageal Cancer; Papillon-Lefèvre syndrome, Schopf-Schulz-Passarge syndrome), which may be associated with an increased risk of malignancies, especially of the esophagus or colon.

Basaran E, Yilmaz E, Alpsoy E, et al: Keratoderma, hypotrichosis and leukonychia totalis: A new syndrome? Br J Dermatol 133:636, 1995.  [PubMed: 7577599]

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