Ectodermal dysplasia- hypohidrotic- with immune deficiency
A rare genetic disorder characterized inability to sweat, immune system problems and hair and teeth abnormalities.
* Immunodeficiency * Dysgammaglobulinemia * Recurring infections * Frequent pneumonia * Frequent bacterial infections * Low immunoglobulin level * Inability to sweat * Missing secondary teeth * Conical front teeth * Sparse scalp hair * Premature death * Failure to thrive * Frequent respiratory tract infections
Diagnosis is usually by clinical observation often with the assistance of family medical histories so that it can be determined whether transmission is autosomal dominant or recessive.
Prognosis of Ectodermal dysplasia, hypohidrotic, with immune deficiency: infections often leads to death during childhood