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[Alagille's syndrome. Clinical and histo-pathologic study]
Annales De Dermatologie Et De Venereologie
|January 1, 1984
Summary
Alagille syndrome involves chronic liver issues, developmental delays, and physical abnormalities. This review examines skin manifestations and their potential link to zinc deficiency.
Area of Science:
- Hepatology
- Genetics
- Dermatology
Background:
- Alagille syndrome, identified in 1970, is a genetic disorder.
- It is characterized by chronic intrahepatic cholestasis due to bile duct hypoplasia.
Observation:
- The syndrome presents with vertebral and visceral malformations.
- Affected individuals exhibit retarded physical, mental, and sexual development.
- Neonatal jaundice is a common clinical feature.
Findings:
- This review details the clinical, histological, and ultrastructural aspects of cutaneous manifestations in Alagille syndrome.
- The study discusses the potential relationship between Alagille syndrome and zinc deficiency syndrome.
Implications:
- Understanding the cutaneous manifestations can aid in diagnosis and management.
- Investigating the link to zinc deficiency may reveal novel therapeutic targets.