Nasal Deformity in Anhidrotic Ectodermal Dysplasia

Among the various dysplasias, or defects, of the ectodermal appendages, Goeckermann1differentiated the syndrome of hereditary ectodermal dysplasia of the anhidrotic type as a definite clinical entity.2Early cases of this condition were described by Thurnam3in 1848 and by Guilford4in 1883. In 1938 Lord and Wolfe5listed about 50 cases collected from the world literature. However, most of the authors have agreed that the small number of cases reported is due not so much to the rarity of the syndrome as to the unfamiliarity of members of the medical profession with the clinical picture. The main symptoms of this condition are dysplasias of the ectodermal appendages—the sweat glands, hair, teeth and nails—resulting in anhidrosis, hypotrichosis, anodontia and dystrophy of the nails. The other symptoms which are most consistently observed are deformity of the nose and papillary lesions of the skin. Because of the nasal deformity and protruding supraorbital