Jadassohn-Lewandowski Syndrome definition - medical term
This syndrome is a form of what is called
elephant nails from birth (pachyonychia congenita).
The characteristic features include:
Abnormally thick curved nails (onychogryposis)
Thickening of the skin (hyperkeratosis) of the palms, soles, knees and elbows
White plaques (leukoplakia) in the mouth
Excess sweating (hyperhidrosis) of the hands and feet
Teeth are already erupted at birth (natal teeth)
Generation after generation in a family may show the syndrome. It is an autosomal
dominant trait. The gene responsible for the syndrome is on chromosome 12 (in band
12q13) and a single copy of the gene (named PD1) is capable 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 the Jadassohn-Lewandowski type and pachyonychia congenita with natal
teeth and type 1 pachyonychia congenita. The syndrome is named for the professor
of dermatology at the University of Bern in Switzerland, Josef Jadassohn (1860-1936),
and his colleague, Felix Lewandowski (1879- 1921), who first described the syndrome
in 1906. About their patient, a 15-year-old girl, they wrote: "The nail plates of
all the fingers and toes are extremely thickened, and so hard that they cannot be
cut with a scissors; the father has to trim them with a hammer and chisel."
Common Misspellings: jadassohn-lewandowski syndrone
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