Baird syndrome

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Classification according to ICD-10
Q82.8 Other specified congenital skin malformations
ICD-10 online (WHO version 2019)

The Baird's syndrome is a very rare inherited disease with a combination of ectodermal dysplasia and lack of dermal ridges .

Synonyms are: English Ectodermal Dysplasia, Absent Dermatoglyphic Pattern, Changes in Nails, and Simian Crease ; the outdated term is Basan's syndrome or Baasan's syndrome .

The name refers to the first author of the first description from 1964 by HW Baird .

By M. Bashan there is another observation from the year 1965th

distribution

The frequency is given as less than 1 in 1,000,000, inheritance is autosomal dominant .

Clinical manifestations

Clinical criteria are:

  • Onset in newborns
  • Blistering
  • Papules on the face
  • Lack of skin ridges on hands and feet

In addition to flexion contractures of the fingers and toes and membranous syndactyly come.

Individual evidence

  1. ↑ a b c Baird's syndrome. In: Orphanet (Rare Disease Database).
  2. ↑ ADERMATOGLYPHIA WITH CONGENITAL FACIAL MILIA AND ACRAL BLISTERS, DIGITAL CONTRACTURES, AND NAIL ABNORMALITIES.  In: Online Mendelian Inheritance in Man . (English)
  3. ↑ Basan's syndrome. In: Orphanet (Rare Disease Database).
  4. ↑ Who named it
  5. ↑ HW BAIRD: KINDRED SHOWING CONGENITAL ABSENCE OF THE DERMAL RIDGES (FINGERPRINTS) AND ASSOCIATED ANOMALIES. In: The Journal of Pediatrics . Vol. 64, May 1964, pp. 621-631, PMID 14148996 .
  6. ↑ M. Basan: Ectodermal dysplasia without papillary muscles. Nail changes and four-finger furrow. In: Archives for Clinical and Experimental Dermatology . Vol. 222, No. 6, July 1965, pp. 546-557, PMID 5846547 .

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