Case history
A 20-year-old man presents with jaundice that started 2 days after the onset of sore throat, fever, and chills. He reports a history of intermittent jaundice over the last 5 years. Symptoms of fever, chills, and sore throat have resolved with penicillin therapy, but the jaundice has persisted. He has no other past medical history, but a family member has experienced similar episodes of intermittent jaundice.
Other presentations
Patients sometimes report vague abdominal complaints and fatigue, although this does not seem to represent any serious pathology and may be related to anxiety and exhaustive investigations for jaundice.[1]Dubin IN, Johnson FB. Chronic idiopathic jaundice with unidentified pigment in the liver cells: a new clinicopathologic entity with report of 12 cases. Medicine. 1954 Sep;33(3):155-97.
http://www.ncbi.nlm.nih.gov/pubmed/13193360?tool=bestpractice.com
[2]Sprinz H, Nelson RS. Persistent nonhemolytic hyperbilirubinemia associated with lipochrome-like pigment in liver cells: report of 4 cases. Ann Intern Med. 1954 Nov;41(5):952-62.
http://www.ncbi.nlm.nih.gov/pubmed/13208040?tool=bestpractice.com
[3]Dubin IN. Chronic idiopathic jaundice: a review of 50 cases. Am J Med. 1958 Feb;24(2):268-92.
http://www.ncbi.nlm.nih.gov/pubmed/13508683?tool=bestpractice.com
Although hepatomegaly is sometimes seen, liver function tests including bile acids are normal and there is no evidence of hemolysis.[1]Dubin IN, Johnson FB. Chronic idiopathic jaundice with unidentified pigment in the liver cells: a new clinicopathologic entity with report of 12 cases. Medicine. 1954 Sep;33(3):155-97.
http://www.ncbi.nlm.nih.gov/pubmed/13193360?tool=bestpractice.com
[3]Dubin IN. Chronic idiopathic jaundice: a review of 50 cases. Am J Med. 1958 Feb;24(2):268-92.
http://www.ncbi.nlm.nih.gov/pubmed/13508683?tool=bestpractice.com
[4]Javitt NB, Kondo T, Kuchiba K. Bile acid secretion in Dubin-Johnson syndrome. Gastroenterology. 1978 Nov;75(5):931-2.
http://www.ncbi.nlm.nih.gov/pubmed/700338?tool=bestpractice.com
Serum bilirubin often fluctuates and occasionally bilirubin measurements may be within normal limits. By contrast with syndromes associated with true cholestasis, there is no pruritus.[5]Rastogi A, Krishnani N, Pandey R. Dubin-Johnson syndrome: a clinicopathologic study of twenty cases. Indian J Pathol Microbiol. 2006 Oct;49(4):500-4.
http://www.ncbi.nlm.nih.gov/pubmed/17183837?tool=bestpractice.com
[6]Junge N, Goldschmidt I, Wiegandt J, et al. Dubin-Johnson Syndrome as differential diagnosis for neonatal cholestasis. J Pediatr Gastroenterol Nutr. 2021 May 1;72(5):e105-11.
https://www.doi.org/10.1097/MPG.0000000000003061
http://www.ncbi.nlm.nih.gov/pubmed/33534365?tool=bestpractice.com
The disease can develop gradually or acutely, often triggered by an intercurrent illness, alcohol, oral contraceptives, or pregnancy.[5]Rastogi A, Krishnani N, Pandey R. Dubin-Johnson syndrome: a clinicopathologic study of twenty cases. Indian J Pathol Microbiol. 2006 Oct;49(4):500-4.
http://www.ncbi.nlm.nih.gov/pubmed/17183837?tool=bestpractice.com
A high frequency of fetal loss has been reported in women with DJS, although the reasons for this association are not clear.[7]Seligsohn U, Shani M. The Dubin-Johnson syndrome and pregnancy. Acta Hepatogastroenterol. 1977 Jun;24(3):167-9.
http://www.ncbi.nlm.nih.gov/pubmed/883465?tool=bestpractice.com
[8]Di Zoglio JD, Cardillo E. The Dubin-Johnson syndrome and pregnancy. Obstet Gynecol. 1973 Oct;42(4):560-3.
http://www.ncbi.nlm.nih.gov/pubmed/4742659?tool=bestpractice.com
Chronic cholecystitis and cholelithiasis have been linked, but this may be a coincidental finding.[5]Rastogi A, Krishnani N, Pandey R. Dubin-Johnson syndrome: a clinicopathologic study of twenty cases. Indian J Pathol Microbiol. 2006 Oct;49(4):500-4.
http://www.ncbi.nlm.nih.gov/pubmed/17183837?tool=bestpractice.com
[9]Skornick YG, Klausner JM, Lelcuk S, et al. Cholelithiasis in Dubin-Johnson syndrome. Surg Gynecol Obstet. 1983 Nov;157(5):447-9.
http://www.ncbi.nlm.nih.gov/pubmed/6635916?tool=bestpractice.com
[10]Sotelo-Avila C, Danis RK, Krafcik J, et al. Cholecystitis in a 17-year-old boy with recurrent jaundice since childhood. J Pediatr. 1988 Apr;112(4):668-74.
http://www.ncbi.nlm.nih.gov/pubmed/3351696?tool=bestpractice.com