Alagille syndrome

A rare syndrome variably characterized by chronic cholestasis due to paucity of intrahepatic bile ducts, peripheral pulmonary artery stenosis, vertebrae segmentation anomalies, characteristic facies, posterior embryotoxon/anterior segment abnormalities, pigmentary retinopathy, and dysplastic kidneys.

Lichenification

Thickening and hardening of the epidermis seen with exaggeration of normal skin lines.


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PMID (PMCID)
1895727
 MALE Adult
[Keratoconus in Alagille syndrome].
Lang GE, Naumann GO.
Klin Monbl Augenheilkd. 1991;198(6):555-7.
We report on a 20 year old white male with growth retardation, secondary hypothyroidism, typical facies, skin lichenification and brachydactylia due to arteriohepatic dysplasia.