Bonnevie-ullrich syndrome - Altmeyers Encyclopedia

Synonym(s)

Pterygium Syndrome; Status Bonnevie-Ullrich; Ullrich Bonnevie Syndrome

History
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Ullrich, 1930; Bonnevie, 1934

Definition
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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.

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Manifestation
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From birth.

Clinical features
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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.

Therapy
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Symptomatic. Possibly plastic surgery corrections.

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