Bonnevie-ullrich syndromeQ87.16

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Pterygium Syndrome; Status Bonnevie-Ullrich; Ullrich Bonnevie Syndrome

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HistoryThis section has been translated automatically.

Ullrich, 1930; Bonnevie, 1934

DefinitionThis section has been translated automatically.

Rare combination of multiple, predominantly ectodermal malformations with the leading symptom pterygias of the skin ( pterygias of the skin) on neck and joints. Mode of inheritance unexplained.

ManifestationThis section has been translated automatically.

From birth.

Clinical featuresThis section has been translated automatically.

Single and double-sided pterygias, especially of the neck, but also over joints. Lymphedema of the backs of hands and feet. Malformations of the skeletal system (syndactylia, congenital hip luxation, high palate, disorders of longitudinal growth, mandibular hypoplasia). Hypertelorism, auricular malformations. Cutis laxa, hyperextensibility of the joints, increased vulnerability of the skin. Disturbances of the cranial nerve function. In addition, heart malformations, situs inversus, hypospadias, nail dystrophies possible.

TherapyThis section has been translated automatically.

Symptomatic. Possibly plastic surgery corrections.

LiteratureThis section has been translated automatically.

  1. Bonnevie K (1934) Embryological analysis of gene manifestation in Littles ans Baggs abnormal mouse tribe. J Exp Zool 67: 443-520
  2. Ullrich O (1930) On typical combination pictures of multiple varieties. Z Pediatrics (Berlin) 49: 271-276

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer