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An autosomal recessive syndrome consisting of xeroderma pigmentosum associated with severe neurological and developmental involvement. In addition to the clinical signs of xeroderma pigmentosum, patients present with mental retardation, dwarfism, gonadal hypoplasia, microcephaly and various neurologic complications of early onset.




Xerodermic idiocy
Xerodermic idiocy of de Sanctis and Cacchione


Xeroderma pigmentosum
Mental retardation


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