[Anodontia and hidrotic ectodermal dysplasia, hyporeactive to heat. Effect of acitretin]

We observed an adult female patient presenting an autosomal recessive ectodermal dysplasia difficult to classify among the various reported forms of this disease. She had the major signs of the hidrotic type of the disease (palmo-plantar hyperkeratosis, nail and hair dystrophies, and typical sweat glands) and anodontia as reported in the hypohidrotic form of the syndrome. We evaluated the function of the sweat glands and found them hyporeactive to a heat stimulus. Most of the epithelial alterations have improved under therapy by acitretin.