Hypohidrotic Ectodermal Dysplasia with Immune Deficiency - Key Clinical Features

• Severe recurrent infections - respiratory tract, skin, soft tissues, bones, GI tract
• Meningitis and septicaemia may occur in early childhood
• Failure to thrive
• Lower-than-normal number of teeth
• Delayed or absent tooth formation
• Inability to sweat
• Absent tears (occasional)
• Thin skin
• Decreased skin pigment
• Foul-smelling nasal discharge
• Poor temperature regulation
• Heat intolerance
• Scanty hair
• Absent hair
• Abnormal nails
• Low nasal bridge