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Hidrotic Ectodermal Dysplasia


Autosomal dominant disorder characterized by nail dystrophy associated with defects of the hair and palmoplantar keratoderma. The nails are thickened, striated, often discoloured and grow slowly. Paronychial infections are frequent and may partially or completely destroy the matrix. Diffuse hyperkeratosis of the palms and occurs frequently and may be severe. In the complete forms scalp hair is very sparse, fine and brittle and is usually absent after puberty.




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