Hereditary Dystrophy of the Hair and Nails

Ectodermal Dysplasias Revisited

“…The same kind of variability is found as regards onychodysplasia. Weech (1929), for instance, had already noted that the nail defect of the condition described and reviewed by Jacobsen (1928) was never seen in his " anhidrotic form ". What then is an ectodermal dysplasia ?…”

Clouston syndrome: an ultrastructural study

“…Clouston Syndrome (CS) is a form of Ectodermal Dysplasia (ED), first described in 1895 by Nicolle & Halliprt. Since that time, a few families with multiple affecteds have been reported (White 1896, Hoffman 1908, Eisenstaedt 19 13, Barrett 19 19, Tobias 1925, Mayer 1928, Thompson 1928, Jacobsen 1928, Clouston 1929, Nelson 1931, Joachim 1936, Clouston 1939, Wilkey & Stevenson 1945. Constant features of the syndrome include thickening, deformity, hypoplasia, and occasional absence of nails.…”

Incidence of dystrophies caused by fluorine in organs regulated by the parathyroid glands

“…4). Whereas the former has, so far as I know, never before been thought of as a dystrophy due to fluorine poisoning, the latter has been described as a condition frequently co-existent with misshapen teeth and with koilonychia (Weech, 1929), and with dystrophies of the nails and hair (Jacobsen, 1928;Clouston, 1929;Hill, 1933), and attributed to endocrine disturbances or to a congenital ectodermal defect of unknown origin (Goeckermann, 1920;MacKee & Andrews, 1924). 20 % of the people examined in the present series exhibited one or the other of these dental dystrophies.…”

“…None of these, however, appears to have ever made any suggestion as to the cause of either the dermatoses concerned, or of the onychodystrophies, or of the co-existence of both these lesions. But when the co-existent lesions of the skin and nails were found to be accompanied by those of the hair and teeth (Thurnam, 1848;MacKee & Andrews, 1924;Tobias, 1925: Jacobsen, 1928) the possibility of these trophic changes being due to endocrine deficiency was mentioned, but the suspicion that the parathyroid glands might be at fault has hardly ever arisen. It should, however, be remembered that fluorine as an aetiological factor producing mottled teeth, through the parathyroid glands being primarily affected in a manner analogous with that in which Erdheim produced dental lesions by means of parathyroidectomy (1906), has been recognized by Bergara (1927), Chaneles (1929b) and Pavlovic & Tihomirov (1932).…”