Hypohidrotic ectodermal dysplasia with atrophic rhinitis and nasal myiasis

Int J Pediatr Otorhinolaryngol. 2009 Oct;73(10):1467-9. doi: 10.1016/j.ijporl.2009.06.012. Epub 2009 Jul 18.


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.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple / diagnosis*
  • Anti-Bacterial Agents / therapeutic use
  • Child
  • Ectodermal Dysplasia, Hypohidrotic, Autosomal Recessive / diagnosis*
  • Ectodermal Dysplasia, Hypohidrotic, Autosomal Recessive / genetics
  • Endoscopy / methods
  • Humans
  • Infusions, Intravenous
  • Male
  • Myiasis / diagnosis*
  • Myiasis / drug therapy
  • Nose Diseases / diagnosis
  • Prognosis
  • Rare Diseases
  • Rhinitis, Atrophic / diagnosis*
  • Rhinitis, Atrophic / pathology
  • Risk Assessment
  • Tomography, X-Ray Computed / methods


  • Anti-Bacterial Agents