Hostname: page-component-cd9895bd7-gvvz8 Total loading time: 0 Render date: 2024-12-26T22:20:29.471Z Has data issue: false hasContentIssue false
  • English
  • Français

Persistent nasal crusting due to hypohidrotic ectodermal dysplasia

Published online by Cambridge University Press:  29 June 2007

A. H. Al-Jassim  and
A. C. Swift
A. H. Al-Jassim
Affiliation:
Department of Otorhinolaryngology, Aintree Hospitals (Walton), Rice Lane, Liverpool, UK
A. C. Swift*
Affiliation:
Department of Otorhinolaryngology, Aintree Hospitals (Walton), Rice Lane, Liverpool, UK
*
Address for correspondence: A. C. Swift, Ch.M., F.R.C.S., F.R.C.S.Ed., Consultant Otorhinolaryngologist, Department of ENT Surgery, Aintree Hospitals (Walton), Rice Lane, Liverpool L9 1AE. Fax: 0151 529 4782
Get access Rights & Permissions [Opens in a new window]

Abstract

Hypohidrotic ectodermal dysplasia is an hereditary condition of the ectodermal tissues which may escape recognition because of lack of clinical awareness due to its rarity. Otorhinolaryngological features of this syndrome include chronic respiratory tract infections, persistent foul-smelling nasal discharge and crust formation, and hearing problems.

The condition is usually an X-linked recessive disease affecting mainly ectodermal tissue, although nonectodermal tissue may also be affected. The notation ‘ectodermal’ is used because ectodermal tissues are always involved. The syndrome is characterized by complete or partial absence of sweat glands, sparse hair growth, absent or deformed peg teeth, sparse sebaceous glands, occasional absence of salivary and lacrimal glands, scanty mucous glands and deficient cilia.

We describe two children with this disorder with specific reference to the effect on the upper respiratory tract.

Keywords

Ectodermal dysplasia
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 110 , Issue 4 , April 1996 , pp. 379 - 382
Copyright
Copyright © JLO (1984) Limited 1996

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Angelina Mariano, M., Blanchet Bardon, C. L. (1990) Diagnostico antenatal E.M. dermatologia: contribuicao do estudo ultraestrutural. Ada Medica Portuguesa 2: 101–107.Google Scholar
Beahrs, J. O., Lillington, G. A., Rosan, R. C., Russin, L., Lindgren, J. A., Rowley, P. T. (1971) Anhidrotic ectodermal dysplasia: Predisposition to bronchial disease. Annals of Internal Medicine 74: 92–96.CrossRefGoogle ScholarPubMed
Bennett, C. G. (1963) Study of exfoliative cytology of oral mucosa of children exhibiting clinical evidence of ectodermal dysplasia. Journal of Dental Research 4: 943–949.Google Scholar
Brodie, A. G., Sarnat, B. G. (1942) Ectodermal dysplasia (anhidrotic type) with complete anodontia. A serial roentgenographic cephalometric appraisal. American Journal of Diseases of Children 64: 1046–1054.Google Scholar
Clarke, A., Philips, D. I. M., Brown, R., Harper, P. S. (1987) Clinical aspects of X-linked hypohidrotic ectodermal dysplasia. Archives of Disease in Childhood 62: 989–996.Google Scholar
Clouston, H. R. (1939) The major forms of hereditary ectodermal dysplasia. Journal of the Canadian Medical Association 40: 1.Google ScholarPubMed
Darwin, C. (1890) The Variations of Plants and Animals Under Domestications, vol 2, D. Appleton and Company Inc., New York, p 319.Google Scholar
Everett, F. G., Jump, E. B., Sutherland, W. F., Savara, B. S., Suher, T. (1952) Anhidrotic ectodermal dysplasia with anodontia. A study of two families. Journal of the American Dental Association 173–186.CrossRefGoogle ScholarPubMed
Felsher, Z. (1944) Hereditary ectodermal dysplasia. Report of a case with experimental study. Archives of Dermatology and Syphilology 410–414.CrossRefGoogle Scholar
Freire-Maia, N., Pinheiro, M. (1984) Ectodermal Dysplasia. A Taxonomic Approach in Ectodermal Dysplasias: A Clinical and Genetic Study. 1st Edition, Liss A. R., Inc., New York, p 22–27.Google Scholar
Hartwell, S. W. Jr, Pickrell, K., Quinn, G. (1965) Congenital anhidrotic ectodermal dysplasia. A report of two cases. Clinical Pediatrics 4: 383–386.CrossRefGoogle ScholarPubMed
Helweg-Larsen, H. J., Ludvigsen, K. (1946) Congenital familial anhidrosis and neurolabyrinthitis. Ada Dermato-Venereologica (Stockholm) 26: 489–505.Google ScholarPubMed
Lipton, I., Roberts, M. H. (1950) Hereditary ectodermal dysplasia of the anhidrotic type. American Journal of Diseases in Children 79: 504–509.Google Scholar
Lowry, B. B., Robinson, G. G., Miller, J. R. (1966) Hereditary ectodermal dysplasia symptoms, inheritance patterns, differential diagnosis, management. Clinical Pediatrics 5: 395–402.Google Scholar
Metson, B. F., Williams, B. K. (1952) Hereditary ectodermal dysplasia of the anhidrotic type. Report of a case in a negro. Journal of Pediatrics 40: 303–309.Google Scholar
Reed, W. B., Lopez, D. A., Landing, B. (1970) Clinical spectrum of anhidrotic ectodermal dysplasia. Archives of Dermatology 102: 134–143.CrossRefGoogle ScholarPubMed
Rozycka-Chrzanowska, B., Hryniewiecki, T., Solik-Tomassi, A., Rawczynska-Englert, I., Sliwinski, M., Pajewska-Klimczak, M. (1994) Mitral and aortic valve replacement in a patient with ectodermal anhydrotic dysplasia. A case report. Journal of Heart Valve Disease 3: 224–225.Google Scholar
Shaw, R. M. (1990) Prosthetic management of hypohydrotic ectodermal dysplasia with anodontia. Case report. Australian Dental Journal 35: 113–116.CrossRefGoogle ScholarPubMed
Sunderman, F. W. (1941) Persons lacking sweat glands. A hereditary ectodermal dysplasia of the anhidrotic type. Archives of Internal Medicine 846–854.Google Scholar
Thurnam, J. (1848) Two cases in which the skin, hair and teeth were very imperfectly developed. Medicochirurgical Transactions 71–82.Google ScholarPubMed
Upshaw, B. Y., Montgomery, H. (1949) Hereditary anhidrotic ectodermal dysplasia. A clinical and pathological study. Archives of Dermatology and Syphilology 60: 1170–1183.Google Scholar
Weech, A. A. (1929) Hereditary ectodermal dysplasia. (Congenital ectodermal defect). A report of two cases. American Journal of Diseases in Children 37: 766–789.CrossRefGoogle Scholar