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Anais Brasileiros de Dermatologia
On-line version ISSN 1806-4841
SILVA, Cláudia Márcia Resende; BAHIA, Magda and GONTIJO, João Renato Vianna. Do you know this syndrome?. An. Bras. Dermatol. [online]. 2008, vol.83, n.3, pp. 265-268. ISSN 1806-4841. http://dx.doi.org/10.1590/S0365-05962008000300012.
Alagille syndrome, an inherited autosomal disorder of dominant trait, is one of the most common causes of chronic liver disease in childhood. Its hallmark is the paucity of intrahepatic bile ducts, with variable degrees of liver impairment. Cutaneous manifestations include intense pruritus, often refractory to treatment, and xanthomata.
Keywords : Alagille syndrome; Cholestasis intrahepatic; Pruritus; Xanthomatosis.