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.

Comedo

A clogged cutaneous sebaceous follicle, which is a cutaneous gland that secretes sebum (usually into a hair follicle).


Total: 2

                       


(per page)
PMID (PMCID)
9536240
 MALE Child
Steatocystoma multiplex and leuconychia in a child with Alagille syndrome.
Cambiaghi S, Riva S, Ramaccioni V, Gridelli B, Gelmetti C.
Br J Dermatol. 1998;138(1):150-4.
Cutaneous involvement in this disorder is quite rare and the association of Alagille syndrome with multiple comedones and cysts has been described only once.
9536240
 MALE Child
Steatocystoma multiplex and leuconychia in a child with Alagille syndrome.
Cambiaghi S, Riva S, Ramaccioni V, Gridelli B, Gelmetti C.
Br J Dermatol. 1998;138(1):150-4.
Here, we report the clinical and histological findings of a 7-year-old patient affected by Alagille syndrome who presented multiple cystic lesions and comedones reminiscent of steatocystoma multiplex and a congenital total true leuconychia.