Journal of Nepal Medical Association (Nov 2024)

Nasal Myiasis in a Female with Christ–Siemens–Touraine Syndrome: A Case Report

  • Leison Maharjan,
  • Anju Shah,
  • Dhirendra Yadav,
  • Namita Shrestha

DOI
https://doi.org/10.31729/jnma.8848
Journal volume & issue
Vol. 62, no. 280

Abstract

Read online

Ectodermal dysplasia is a rare disease that belongs to a diverse group of inherited monogenic disorders involving defects in one or more ectodermally or mesodermally derived tissues. Hypohidrotic ectodermal dysplasia, also known as Christ-Siemens-Touraine syndrome is a type of ectodermal dysplasia characterized by a triad of anhidrosis, dysodontia, and hypotrichiasis. The most prevalent method of transmission is X-linked recessive, manifesting fully in men and only partially in female carrier heterozygotes. Atrophic rhinitis and nasal myiasis are rare characteristics of this condition. We hereby report a case of a 52-year-old female with atrophic rhinitis and nasal myiasis who was managed conservatively.

Keywords