CHRIST SIEMENS TOURAINE SYNDROME WITH NASAL MYIASIS SECONDARY TO ATROPHIC RHINITIS

Ectodermal Dysplasias (ED), hypohidrotic ectodermal dysplasia, a rare recessive genetic disease linked to chromosome, is characterized by heat intolerance, excessively dry skin due to the absence of sweat glands and abnormal spiky or absent teeth7,9. It is caused by mutation in a movel transmenbrane protein, ectodysplasin A2, mainly involving ectodermal structures such as epidermis and its annexes (hair and nails), although nonectodermal tissue may also become involved3,8. Otolaryngological manifestations are related to hypoplasia of the mucous glands of the upper aerodigestive tract such as rhinitis, pharyngitis, bronchitis and otitis, and also epistaxis, dysphagia, anodontia and ozena.