Congenital Ectodermal Dysplasia

THREE cases of congenital ectodermal dysplasia have been encountered in schools for partially sighted children in the London area.The features of this syndrome are widespread and include the following: Scarcity of hair growth, which is most conspicuous on the scalp; Abnormality or absence of the teeth and nails; Absence of the mucous glands of the nose leading to chronic rhinitis, and dryness of the skin;Skeletal abnormalities, including depression of the bridge of the nose and underdevelopment of the lower jaw.The ocular defects associated with the condition are congenital cataracts, which were originally present in two of these cases, and were removed by needlings.In the other case chorioretinal disturbances are present.Two out of the three cases are females, which is unusual, since it is rare for females to be severely affected by this disorder.