Premature aging syndromes affecting the skin.
Consideration of the above syndromes from a dermatologic perspective leads to several conclusions: 1) The "normal changes" associated with aging of the skin have been inadequately defined to date. 2) Although all the premature aging syndromes manifest certain features suggestive of accelerated aging, none closely mimics the normal aging process. The distribution, specific character, and developmental sequence of pathologic findings diverge from those of normal aging for most organ systems in each syndrome. Furthermore, in each disorder many of the accepted features of normal aging are lacking. These discrepancies are at least as pronounced in the skin as in the other involved organ systems. 3) Most of the premature aging syndromes are transmitted by autosomal recessive genes, yet have multiple features that are difficult or impossible to attribute to a single defective enzyme or other protein structure. This suggests that the syndromes may be due to errors in the regulation of various metabolic pathways and argues in favor of an explicit genetic program for aging. 4) The frequency and variety of cutaneous abnormalities in these complex syndromes emphasize the integral relationship of the skin to the rest of the body and the potential value of the skin as a tissue source for studies of underlying disease mechanisms. 5) The relatively recent application of tissue culture technology to the study of progeria, Werner syndrome, and the other premature aging syndromes has already yielded considerable data, offering the hope that our understanding of these diseases may increase rapidly in the coming decades.
D000328:Adult; D000375:Aging; D003094:Collagen / Q000378:metabolism; D004260:DNA Repair; D004314:Down Syndrome / Q000473:pathology; D005260:Female; D005347:Fibroblasts / Q000201:enzymology / Q000473:pathology; D006801:Humans; D066298:In Vitro Techniques; D008297:Male; D011371:Progeria / Q000378:metabolism / Q000473:pathology; D012867:Skin / Q000378:metabolism / Q000473:pathology*; D014898:Werner Syndrome / Q000473:pathology
D003094:Collagen