Ectodermal dysplasia with alopecia, onychodysplasia, hypohidrosis, keratoderma, abnormal teeth and deafness.

The ectodermal dysplasias are a heterogeneous group of disorders with primary defect in hair, teeth, nail and sweat gland function. Numerous types have been described and several classifications exist. Here, we present a patient with ectodermal dysplasia with alopecia, dysplastic nails, hypohidrosis, sensorineural deafness, palmoplantar keratoderma, abnormal teeth and dry skin. To our knowledge, combination of all these features in ectodermal dysplasia has not been reported in the past. The etiology is unknown, but consanguinity of parents points to an autosomal recessive inheritance.

Sindrome da unha amarela: a proprósito de um caso com derrame pericárdico / Yellow mail syndrome: apropos of a case with pericardial effusion

A síndrome da unha amarela com preende três elementos: edema linfático, alteraçöes ungueais e manifestçöes pleuropulmonares. No caso relatado o quadro clínico característico é acompanhado de derrame pericárdico, até hoje näo descrito nesta síndrome