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Abstract

French

Background:

Offspring resulting from consanguineous marriages have been important in advancing research in genodermatoses.

Objective:

The concepts of consanguinity and inbreeding are reviewed and their contributions to research in hereditary skin diseases explained.

Methods:

Examples are given in which inbred individuals with hereditary skin disease have increased our knowledge of skin disease genetics.

Results:

An enhanced understanding of the genetics of xeroderma pigmentosum, lamellar ichthyosis, and Werner's syndrome, among others, has arisen from the observation of populations and families in which inbreeding has occurred.

Conclusion:

Populations practicing inbreeding and consanguineous marriages in generally outbred populations have provided important observations for determining the inheritance pattern of several genodermatoses and for identifying the responsible genes.

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Geneticists Share the Family Jewels: How Inbreeding Has Contributed to Understanding Hereditary Skin Disease