Hereditary Ectodermal Dysplasia of the "Anhydrotic Type"

In 1838 Widderburn reported a very striking anomaly of the skin which he observed in ten males of a Hindu family. The symptomatic triad of this anomaly consisted of complete inability to sweat (anhydrosis), a deficiency of the scalp, axillary and pubic hair (hypotrichosis) and partial absence or an incomplete development of the teeth (anadontia). In recent years Thadani has reported similar cases occurring in Hindu families from a district where inbreeding is common. Among Europeans Thurman in 1848, Williams in 1848, Guilford in 1883 and Hutchinson in 1886 described similar hereditary ectodermal anomalies. Guilford in 1883 reported the existence in his patient of a saddle nose and established the fact that the afflicted members of the patient's family had the same deformity of the nose. Hutchinson observed a defect on the mamilla, a finding that is not present in all cases. In the German literature Tendlau in 1902. Weichselmann