1/3. Anhidrotic ectodermal dysplasia presenting as atrophic rhinitis.Atrophic rhinitis is a chronic inflammatory disease of the nose. The aetiology of primary atrophic rhinitis is not yet known, although secondary atrophic rhinitis is known to be associated with chronic granulomatous diseases such as tuberculosis and leprosy. The authors report a case of atrophic rhinitis, which was a presenting feature of a rare genetic disorder known as Christ-Siemens-Touraine syndrome, also known as anhidrotic ectodermal dysplasia.- - - - - - - - - - ranking = 1keywords = anhidrotic, ectodermal dysplasia, dysplasia (Clic here for more details about this article) |
2/3. Atrophic rhinitis in a patient with anhidrotic ectodermal dysplasia.We would like to present the rare case of a now 37-year old female patient with autosomal-recessively inherited anhidrotic ectodermal dysplasia being treated in our ENT department for atrophic rhinitis. The clinical appearance very much resembled the picture of an "empty nose" with distinct hypoplasia of the turbinates and extensively wide nasal cavities. We want to point out the possible existence of atrophic rhinitis against the background of an underlying syndromatic disease in adults and also the pediatric patient.- - - - - - - - - - ranking = 2.8405782711455keywords = anhidrotic, ectodermal dysplasia, dysplasia (Clic here for more details about this article) |
3/3. Ozena as presenting symptom of a rare and severe genetic disease: hypohidrotic ectodermal dysplasia.Ozena is quite a common clinical finding in ENT practice in many countries, but even if the pathological picture is clear, its etiology is unknown. We report on two young females in which ozena was the presenting symptom of a rare and severe genetic disorder (hypohidrotic ectodermal dysplasia, HED). Etiopathogenetic theories on ozena and genetic problems of HED are discussed.- - - - - - - - - - ranking = 0.53985543221363keywords = ectodermal dysplasia, dysplasia (Clic here for more details about this article) |