Human hereditary nail disorders constitute a rare and heterogeneous group of ectodermal dysplasias. They occur as isolated and/or syndromic ectodermal conditions where other ectodermal appendages are also involved, and can occur associated with skeletal dysplasia. 'Nail disorder, nonsyndromic congenital' (OMIM; Online Mendelian Inheritance in Man) is subclassified into 10 different types. The u...

www.medigraphic.org.mx * Specialist in Endodontics and Dental Radiology and Imaging, Hospital for the Rehabilitation of Craniofacial Anomalies of the University of Sao Paulo. Professor in Applied Dental Sciences, Bauru School of Dentistry, University of Sao Paulo, Brazil. § PhD in Oral Pathology. Bauru School of Dentistry, University of Sao Paulo, Brazil. Teacher at the Pediatric Dentistry Depa...

Ectodermal dysplasia syndromes are frequently associated with manifestations of disease in the respiratory tract. Commonly, patients present with signs and symptoms of sinusitis, otitis media with effusion, bronchitis or pneumonia. The etiologic bases for these disorders are multi-factorial, including abnormal mucous production, anatomical variations, atopy and immune function abnormalities. Th...

Ectodermal dysplasias are conditions that present primary defects in two or more tissues of ectodermal origin and can be classified as hypohidrotic and hidrotic. Hidrotic ectodermal dysplasia or Clouston syndrome is an autosomal dominant genodermatosis and appears as a triad of clinical findings: palmoplantar keratoderma, nail dystrophy, and hypotrichosis. The hair is sparse and brittle. The na...