title: Hypohidrotic ectodermal dysplasia with atrophic rhinitis and nasal myiasis.
abstract: Hypohidrotic/anhidrotic ectodermal dysplasia is a rare inherited disorder characterized by hypohidrosis/anhidrosis, hypotrichosis, dysodontia and heat intolerance. Most common mode of transmission is X-linked recessive, showing complete expression in males, and only partial manifestations in the female carrier heterozygotes. Features like atrophic rhinitis, nasal and aural myiasis, syndactyly, cleft lip and/or palate, mental retardation and immunodeficiency are uncommonly seen in this syndrome. We hereby report a case of hypohidrotic ectodermal dysplasia with unusual features of atrophic rhinitis and nasal myiasis.
authors.affiliation: Department of Otorhinolaryngology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry 605 006, India. maddyy@gmail.com
authors.forename: J
authors.initials: J
authors.lastname: Madana
authors.forename: Deeke
authors.initials: D
authors.lastname: Yolmo
authors.forename: S
authors.initials: S
authors.lastname: Gopalakrishnan
authors.forename: S K
authors.initials: SK
authors.lastname: Saxena
authors.forename: A K
authors.initials: AK
authors.lastname: Nath
authors.forename: V
authors.initials: V
authors.lastname: Ilamaran
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis*; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D002648:Child; D053360:Ectodermal Dysplasia, Hypohidrotic, Autosomal Recessive / Q000175:diagnosis* / Q000235:genetics; D004724:Endoscopy / Q000379:methods; D006801:Humans; D007262:Infusions, Intravenous; D008297:Male; D009198:Myiasis / Q000175:diagnosis* / Q000188:drug therapy; D009668:Nose Diseases / Q000175:diagnosis; D011379:Prognosis; D035583:Rare Diseases; D012222:Rhinitis, Atrophic / Q000175:diagnosis* / Q000473:pathology; D018570:Risk Assessment; D014057:Tomography, X-Ray Computed / Q000379:methods
chemical_list: D000900:Anti-Bacterial Agents