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January 1982

Malignant Melanoma and Papillon-Lefèvre Syndrome

Arch Dermatol. 1982;118(1):2. doi:10.1001/archderm.1982.01650130006003

To the Editor.—  Papillon-Lefèvre syndrome is a rare autosomal recessive genodermatosis characterized by keratosis palmaris et plantaris and periodontosis, which leads to premature loss of both the deciduous and permanent teeth. An increased susceptibility to infections has been described that includes furunculosis, abscesses of the skin and liver, and hidradenitis suppurativa in 21% of the patients. In some patients, arachnodactyly has also been reported. In a comprehensive review by Haneke1 in 1979 of 120 cases reported in the literature, no cases of malignant neoplasms were observed. We describe herein a patient with the Papillon-Lefèvre syndrome in whom malignant melanoma developed.

Report of a Case.—  A 31-year-old man had had the Papillon-Lefèvre syndrome since infancy, with severe dorsal keratosis palmaris et plantaris, periodontosis, abscesses of the skin, furunculosis, and arachnodactyly (proved by roentgenograms). The patient had noticed a lesion present on one finger for almost eight months and had observed

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