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
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