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Pachyonychia congenita, type 1: This is a form of pachyonychia congenita (elephant nails from birth) in which teeth are evident at birth. It is called the Jadassohn-Lewandowski syndrome.
The characteristic features include:
Generation after generation in a family may show the syndrome. It is anautosomal dominant trait. The gene responsible for the syndrome is onchromosome 12 (in band 12q13) and a single copy of the gene (named PD1) iscapable of causing the disease. The basic abnormality is a mutation (change)in a gene for keratin, a primary constituent of nails, hair, and skin.Alternate names for the syndrome include pachyonychia congenita of theJadassohn-Lewandowski type and pachyonychia congenita with natal teeth. Thesyndrome is named for the professor of dermatology at the University ofBern in Switzerland, Josef Jadassohn (1860-1936), and his colleague, FelixLewandowski (1879-1921), who first described the syndrome in 1906. Abouttheir patient, a 15-year-old girl, they wrote: The nail plates of all thefingers and toes are extremely thickened, and so hard that they cannot becut with a scissors the father has to trim them with a hammer and chisel.