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BY JOHN A. PARRISH, HOWARD P. BADEN, LOWELL A. GOLDSMITH AND MXRON H. MATZ Department of Dermatology, Harvard Medical School, and the Massachusetts General Hospital, Boston, Massachusetts 021 14 Hypotrichosis has been described in a number of hereditary syndromes, many of which have obvious congenital defects in skin and other organ systems (Porter & Lobitz, 1970). Striking diffuse hypotrichosis is usually associated with developmental defects of the hair follicle or structural anamdies of the shaft. Rare exceptions t o this have been noted in individuals as a sporadic occurrence (Saint-Paul, 1962 ;Dochao & Monux, 1966) and in one family as a recessively inherited characteristic (Landes & Langer, 1956). Most reports of inherited syndromes of hypotrichosis have only given descriptions of the morphological alterations of the hair structure. I n the case of ectodermal dysplasia, however, several recent studies have been concerned with the mechanical properties (Swanbeck, Nyren & Juhlin, 1970) and chemical composition (Scriver et al. 1965) of the hair, and it has been suggested there may be an abnormality of the structural proteins. The data are open to some criticism and the changes observed could be secondary t o a disorder of hair growth. As yet, pathophysiological
Annals of Human Genetics – Wiley
Published: Mar 1, 1972
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