Benign recurrent intrahepatic cholestasis: late initial diagnosis in adulthood.

[benign recurrent intrahepatic cholestasis]

Benign recurrent intrahepatic cholestasis (BRIC ) is a rare autosomal recessive or sporadic disorder , characterized by recurrent episodes of intense pruritus and jaundice that resolve spontaneously without leaving considerable liver damage . The attacks can start at any age , but the first attack is usually seen before the second decade of life . We report the case of a young adult male patient with BRIC who presented with recurrent cholestatic jaundice and pruritus with negative work up for all possible etiologies and a liver biopsy consistent with intrahepatic cholestasis . He improved on treatment with rifampicin and has not suffered another attack on follow up . Although in adulthood , BRIC diagnosis should be kept in mind in patients with recurrent cholestatic attacks with symptom free intervals after main bile duct obstruction and other congenital or acquired causes of intrahepatic cholestasis excluded .

Diseases
Validation

Diseases presenting "pruritus" symptom

benign recurrent intrahepatic cholestasis

cholangiocarcinoma

cutaneous mastocytosis

dystrophic epidermolysis bullosa

erdheim-chester disease

lamellar ichthyosis

pleomorphic liposarcoma

pyomyositis

zellweger syndrome

This symptom has already been validated