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AUTOSOMAL DOMINANT INHERITANCE OF ALOPECIA CONGENITA WITH KERATOSIS PALMO-PLANTARIS

The syndrome complex of congenital (total) alopecia, keratosis of palms and soles and dystrophy of nails is a rare mutation with an autosomal dominant pattern of inheritance. Only one other report could be found (Stevanovic, Acta Genet. 9:127, 1959). In the previously described kindred, as well as in the present one, there was variable expression of the listed traits. In the present kindred there were three individuals with the full syndrome and five with partial or no alopecia, yet exhibiting keratosis of palms and soles and nail dystrophy. The proposita had total alopecia, including absence of eyebrows, eyelashes and sexual hair. She perspired normally and had no dental abnormalities. Nails were ridged and dystrophic, and palms and soles were covered with heavy keratosis having an opalescent orange-pink color. Intelligence was normal. Karyotype on peripheral leucocytes was 46, XX. Banding studies are incomplete.Similar variability in expression of traits is seen in other vertically inherited syndromes such as pseudo-pseudohypoparathyroidism and Gardner's syndrome, and may represent unequal crossing over with duplication or triplication of loci in subsequent generations. An animal model is represented by the situation at the Bar locus in Drosophila (region 16-A of the X-chromosome). Unequal crossing over results in duplication or triplication of region 16-A, with triple-Bar presenting a more abnormal phenotype than double Bar. Whether present banding techniques are sensitive enough to test this hypothesis in man remains to be seen.