Reactions 900 - 4 May 2002

SCarbamazepine

Vanishing bile duct syndrome and Stevens-Johnsonsyndrome in a child: case report

A 4-year-old boy developed vanishing bile duct and Stevens-Johnson syndromes after receiving carbamazepine therapy forseizures.

The boy had been receiving carbamazepine [dosage notstated] for 4 months when he was admitted to hospital with afever and a rapidly progressive necrotising rash. One monthprior to admission, his serum carbamazepine concentrationwas 8 µg/ml.

On admission, the boy had discrete and confluenterythematous macules over his face, trunk, extremities andgenitalia; he also had erosions and crusting over his lips. Hisabdomen was distended and splenomegaly was noted.Laboratory tests revealed the following levels: total bilirubin 4mg/dl, ALT 673 IU/L, AST 444 IU/L, alkaline phosphatase 481IU/L, γ-glutamyl transpeptidase (GGT) 613 IU/L andcholesterol 200 mg/dl. An abdominal ultrasound revealed aheterogenic liver and a skin biopsy confirmed a diagnosis ofStevens-Johnson syndrome.

Carbamazepine was discontinued and therapy withmethylprednisolone and ursodeoxycholic acid was started.Over the next 2 days, the boy developed leucopenia. A liverbiopsy on day 4 revealed that intrahepatic bile ducts wereinconspicuous with epithelial destruction and intraepitheliallymphocytic infiltrates. The findings were consistent with earlyvanishing bile duct syndrome.

The boy’s pancreatitis, hepatitis and Stevens-Johnsonsyndrome resolved rapidly, but his GGT and cholesterol levelsremained elevated. On day 16, tacrolimus was started and,after one month of triple therapy, a repeat liver biopsy showedresolution of abnormalities. GGT and cholesterol levelsnormalised after 6 weeks.

Author comment: ‘Our patient is the first child reported tohave carbamazepine-associated acute VBDS [vanishing bileduct syndrome] and, to our knowledge, only the third case ofpediatric acute drug-related VBDS in the literature.’Garcia M, et al. Efficacy of early immunosuppressive therapy in a child withcarbamazepine-associated vanishing bile duct and Stevens-Johnson syndromes.Digestive Diseases and Sciences 47: 1771-82, Jan 2002 - USA 800899392

1

Reactions 4 May 2002 No. 9000114-9954/10/0900-0001/$14.95 Adis © 2010 Springer International Publishing AG. All rights reserved