Bureau-Barrière-Thomas Syndrome

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

The Bureau-Barrière Thomas syndrome , also Bureau-Barrière II syndrome or only Bureau syndrome , lat .: Keratodermia palmo-plantar diffuse Bureau-Barrière-Thomas is a very rare congenital increased callus formation of the soles and palms with piston form of Fingers and toes.

The name refers to the authors, the French dermatologists Henri Barrière and Yves Bureau (1900-1993).

The syndrome should not be confused with Bureau-Barrière syndrome .

distribution

Inheritance is autosomal - recessive .

Clinical manifestations

The changes are already noticeable during or shortly after birth. The skin is affected symmetrically and in general, and there is also increased sweating ( hyperhidrosis ).

literature

  • HJ Rauch, K. Neumayer: [Bureau-Barrière-Thomas-Syndrome. A rare hereditary palmoplantar keratoderma with associated symptoms (author's transl)]. In: Journal of Skin Diseases. Volume 56, Number 2, January 1981, pp. 102-108, ISSN  0301-0481 , PMID 6452757 .

Individual evidence

  1. ↑ The Freedictionary
  2. a b B. Leiber: The clinical syndromes. Syndromes, sequences and symptom complexes. Edited by G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7th edition. Urban & Schwarzenberg, 1990, ISBN 3-541-01727-9 .
  3. ^ Y. Bureau, M. Horeau, H. Barrière et al .: Two cases of drumstick fingers with palmoplantar hyperkeratosis and bone lesions. In: Bull Soc Fr Dermatol Syphiligr. 65, 1958, pp. 328-330.
  4. LA Schachner, RC Hansen: Pediatric Dermatology. Mosby, 2003, ISBN 0-323-02611-7 .