Skip to main content Accessibility help
×
Home

Persistent nasal crusting due to hypohidrotic ectodermal dysplasia

  • A. H. Al-Jassim (a1) and A. C. Swift (a1)

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.

Copyright

Corresponding author

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

References

Hide All
Angelina Mariano, M., Blanchet Bardon, C. L. (1990) Diagnostico antenatal E.M. dermatologia: contribuicao do estudo ultraestrutural. Ada Medica Portuguesa 2: 101107.
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: 9296.
Bennett, C. G. (1963) Study of exfoliative cytology of oral mucosa of children exhibiting clinical evidence of ectodermal dysplasia. Journal of Dental Research 4: 943949.
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: 10461054.
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: 989996.
Clouston, H. R. (1939) The major forms of hereditary ectodermal dysplasia. Journal of the Canadian Medical Association 40: 1.
Darwin, C. (1890) The Variations of Plants and Animals Under Domestications, vol 2, D. Appleton and Company Inc., New York, p 319.
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 173186.
Felsher, Z. (1944) Hereditary ectodermal dysplasia. Report of a case with experimental study. Archives of Dermatology and Syphilology 410414.
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 2227.
Hartwell, S. W. Jr, Pickrell, K., Quinn, G. (1965) Congenital anhidrotic ectodermal dysplasia. A report of two cases. Clinical Pediatrics 4: 383386.
Helweg-Larsen, H. J., Ludvigsen, K. (1946) Congenital familial anhidrosis and neurolabyrinthitis. Ada Dermato-Venereologica (Stockholm) 26: 489505.
Lipton, I., Roberts, M. H. (1950) Hereditary ectodermal dysplasia of the anhidrotic type. American Journal of Diseases in Children 79: 504509.
Lowry, B. B., Robinson, G. G., Miller, J. R. (1966) Hereditary ectodermal dysplasia symptoms, inheritance patterns, differential diagnosis, management. Clinical Pediatrics 5: 395402.
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: 303309.
Reed, W. B., Lopez, D. A., Landing, B. (1970) Clinical spectrum of anhidrotic ectodermal dysplasia. Archives of Dermatology 102: 134143.
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: 224225.
Shaw, R. M. (1990) Prosthetic management of hypohydrotic ectodermal dysplasia with anodontia. Case report. Australian Dental Journal 35: 113116.
Sunderman, F. W. (1941) Persons lacking sweat glands. A hereditary ectodermal dysplasia of the anhidrotic type. Archives of Internal Medicine 846854.
Thurnam, J. (1848) Two cases in which the skin, hair and teeth were very imperfectly developed. Medicochirurgical Transactions 7182.
Upshaw, B. Y., Montgomery, H. (1949) Hereditary anhidrotic ectodermal dysplasia. A clinical and pathological study. Archives of Dermatology and Syphilology 60: 11701183.
Weech, A. A. (1929) Hereditary ectodermal dysplasia. (Congenital ectodermal defect). A report of two cases. American Journal of Diseases in Children 37: 766789.

Keywords

Persistent nasal crusting due to hypohidrotic ectodermal dysplasia

  • A. H. Al-Jassim (a1) and A. C. Swift (a1)

Metrics

Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed