Systematisierte Lentiginosis mit kongenitaler Taubheit und diskretem Status dysrhaphicus

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Abstract
The third case of a new neuro-cutaneous syndrome, named after the first describers Capute-Rimoin-Konigsmark and consisting of patchy hyperpigmentation, congenital deafness and discrete dysraphic changes of the skeleton is reported. Despite some interferences with other known neuro-cutaneous diseases, the described combination of symptoms is so well out-lined in its phenomenological character that it justifies the interpretation as a separate syndrome.

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