Hypohidrotic ectodermal dysplasia with atrophic rhinitis and nasal myiasis.
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.
Madana
J
J
Department of Otorhinolaryngology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry 605 006, India. maddyy@gmail.com
Yolmo
Deeke
D
Gopalakrishnan
S
S
Saxena
S K
SK
Nath
A K
AK
Ilamaran
V
V
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
D000900:Anti-Bacterial Agents