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Hidrotic Ectodermal Dysplasias

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Abstract

Ectodermal dysplasias (EDs) represent a large heterogeneous group of different malformations of cutaneous appendages. The lowest common denominator of these EDs is disorders of at least two ectodermal differentiation products [7, 31]: (1) hair anomaly, (2) dental anomaly, (3) onychodysplasia, or (4) sweat gland anomaly. Neuroectodermal and further malformations may occur in addition. The X-linked anhidrotic EDs, characterized by their main feature of hypohidrosis or anhidrosis, are most widely known. They stand out by their relatively constant clinical pattern. Hidrotic EDs are considerably more variable. Their multiplicity is now far from easy to survey. Fifty to 100 different disorders would easily be classified as hidrotic EDs (HEDs) with a broad application of the term ED. Most of these HED types, however, are extremely rare and many are single cases. In addition, many disorders are wrongly classified as EDs (secondary involvement of ectodermal differentiation products due to different complex disorders, combination of keratinization disorders with malformation of only one ectodermal differentiation product, variable affection of ectodermal differentiation products in inborn and acquired metabolic diseases and malformation syndromes, and phenocopies) though the malformation of ectodermal differentiation products — skin, appendages, and teeth — is secondary to other alterations. According to the above-mentioned definition, malformations of only one ectodermal differentiation product in combination with ichthyoses or palmoplantar keratoses as well as variable hair and nail changes due to inborn errors of metabolism are not EDs.

Keywords

  • Hair Shaft
  • Ectodermal Dysplasia
  • Dental Anomaly
  • Nail Change
  • Malformation Syndrome

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References

  1. Altmeyer P, Schindera I (1975) Ein Beitrag zur hidrotischen ektodermalen Dysplasie. Hautarzt 26:631–637

    PubMed  CAS  Google Scholar 

  2. Baughman FA (1971) CHANDS: The curly hair — ankyloblepharon — nail dysplasia syndrome. In: Bergsma D (ed) Birth defects original article series, part XII. Skin, hair and nails, Williams and Wilkins, Baltimore, pp 100–102 (Company Article Series, vol 8)

    Google Scholar 

  3. Clouston HR (1929) A hereditary ectodermal dystrophy. Can Med Assoc J 21:18–31

    PubMed  CAS  Google Scholar 

  4. Cote GB, Adamopoulos D, Pantelakis S (1982) Arthrogryphosis and ectodermal dysplasia. Hum Hered 32:71–72

    PubMed  CrossRef  CAS  Google Scholar 

  5. Curry CJR, Hall BP (1979) Polydactyly, conical teeth, nail dysplasia, and short limbs: a new autosomal dominant malformations syndrome. Birth Defects 15/15B:253

    Google Scholar 

  6. Ellis RWB, Creveld SA (1949) A syndrome characterized by ectodermal dysplasia, Polydactyly, chondro-dysplasia, and congenital morbus cordis. Arch Dis Child 15:65–84

    CrossRef  Google Scholar 

  7. Freire-Maia N (1971) Ectodermal dysplasias. Hum Hered 21:309–312

    PubMed  CrossRef  CAS  Google Scholar 

  8. Fried K (1977) Autosomal recessive hidrotic ectodermal dysplasia. J Med Genet 14:137

    PubMed  CrossRef  CAS  Google Scholar 

  9. Gold RJM, Scriver CR (1971) The characterization of hereditary abnormalities of keratin. Clouston’s ectodermal dysplasia. Birth Defects Orig Art Ser VII: 91–95

    Google Scholar 

  10. Hallermann W (1948) Vogelgesicht und Cataracta congenita. Klin Monatsbl Augenheilkd 113:315–318

    Google Scholar 

  11. Haneke E, Wilsch L (1981) Scanning electron microscopic investigations of the hair in ectodermal dysplasia syndromes. In: Orfanos C, Montagna W, Stüttgen G (eds) Hair research. Springer, Berlin Heidelberg New York, pp 433–435

    Google Scholar 

  12. Jorgensen RJ, Warson RW (1973) Dental abnormalities in the tricho-dento-osseous syndrome. Oral Surg 36:693

    CrossRef  Google Scholar 

  13. Kresbach H, Rauch HJ (1984) Beitrag zur Frage eines tricho-odonto-onychialen Subtyps der ektodermalen Dysplasie. Z Hautkr 59:601–613

    PubMed  CAS  Google Scholar 

  14. Meisel-Stosiek M, Haneke E, Bäurle G (1983) Atypische hidrotische ektodermale Dysplasie mit Hyperpigmentierung (Abstract). ADF-Symposium Genetik und Dermatologie, 1–3 July 1983, Münster/Westfalen, p 11

    Google Scholar 

  15. O’Rourk TR, Bravos A (1969) Ail oculo-dento-digital dysplasia. Birth defects Orig Art Ser V 2:226

    Google Scholar 

  16. Pinheiro M, Pereira LC, Freire-Maia N (1981) A previously undescribed condition: tricho- odonto-onycho-dermal syndrome. A review of the trieho-odonto-onychial subgroup of ectodermal dysplasias. Br J Dermatol 105:371–382

    PubMed  CrossRef  CAS  Google Scholar 

  17. Price VH, Odom RB, Ward WH, Jones FT (1980) Trichothiodystrophy. Sulfur-deficient brittle hair as a marker for a neuroectodermal symptom complex. Arch Dermatol 116:1375–1384

    PubMed  CrossRef  CAS  Google Scholar 

  18. Robinson GC, Miller JR, Bensimon FJ (1962) Familial ectodermal dysplasia with sensory- neural deafness and other anomalies. Pediatrics 30:797–802

    Google Scholar 

  19. Robinson GC, Miller JR, Worth HM (1966) Hereditary enamel hypoplasia: its association with characteristic hair structure. Pediatrics 37:498–502

    PubMed  CAS  Google Scholar 

  20. Salamon T, Milicevic M (1964) Über eine besondere Form der ectodermalen Dysplasie mit Hypohidrosis, Hypotrichosis, Hornhautveränderungen, Nagel- und anderen Anomalien bei einem Geschwisterpaar. Arch Klin Exper Dermatol 220:564–575

    CrossRef  CAS  Google Scholar 

  21. Schöpf E, Schulz H, Passarge E (1971) Syndrome of cystic eyelids, palmoplantar keratosis, hypodontia and hypotrichosis as a possible autosomal recessive trait. In: Bergsma D (ed) Birth defects (Orig Art Ser XII) 8:219–221

    Google Scholar 

  22. Schöpf E (1983) Lidrandzysten, Palmoplantarkeratose, Hypodontie und Hypotrichose: ein rezessiv vererbtes Syndrom. ADF-Symposium Genetik und Dermatologie, 1–3 July 1983, Münster/Westfalen (Abstract), p 8

    Google Scholar 

  23. Senter TP, Jones KL, Sahati N, Nyhan WL (1978) Atypical ichthyosiform erythroderma and congenital neurosensory deafness. A distinct syndrome. J Pediat 92:68

    PubMed  CrossRef  CAS  Google Scholar 

  24. Setieis H, Kramer B, Valcareel M, Einhorn AH (1963) Congenital ectodermal dysplasia of the face. Pediatrics 32:540, 548

    Google Scholar 

  25. Slootweg PJ, Huber J (1984) Hallermann-Streiff syndrome. J Oral Pathol 13:147–154

    PubMed  CrossRef  CAS  Google Scholar 

  26. Stevanovic DV (1959) Alopecia congenita. Acta Genet 9:127–132

    PubMed  CAS  Google Scholar 

  27. Tay CH (1971) Ichthyosiform erythroderma, hair shaft abnormalities, and mental and growth retardation. Arch Dermatol 104:4–13

    PubMed  CrossRef  CAS  Google Scholar 

  28. Wilsch L, Haneke E, Schaidt G (1977) Structural hair abnormalities in hidrotic ectodermal dysplasia (HED). Arch Derm Res 259:101–103

    CrossRef  CAS  Google Scholar 

  29. Wilsch L, Haneke E, Hornstein OP (1977) Über die triehoonychotisehe Subgruppe der hidro- tischen ektodermalen Dysplasie (HED). Klin Pädiat 189:343–345

    CAS  Google Scholar 

  30. Witkop CJ (1965) Genetic diseases of the oral cavity. In: Tiecke RW (ed) Oral pathology. McGraw-Hill, New York

    Google Scholar 

  31. Witkop CJ, Brearley LJ, Gentry WC (1975) Hypoplastic enamel, onycholysis, and hypohidrosis inherited as an autosomal dominant trait. Oral Surg 39:71–86

    PubMed  CrossRef  Google Scholar 

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© 1987 Springer-Verlag Berlin Heidelberg

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Haneke, E. (1987). Hidrotic Ectodermal Dysplasias. In: Happle, R., Grosshans, E. (eds) Pediatric Dermatology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-71524-2_5

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  • DOI: https://doi.org/10.1007/978-3-642-71524-2_5

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-16991-8

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