A Case of Sarcoidosis Presenting as Cirrhosis and Portal Hypertension

Sarcoidosis is a systemic granulomatous disease of unknown etiology that can involve nearly all organs. Liver is the third most commonly affected organ following the lungs and the lymph nodes. Cases with liver sarcoidosis are usually silent clinically while a few can progress to cirrhosis and portal hypertension in less than 1% of the patients.
A 56 year old female was referred for ecchymosis, protuberant abdomen, bilateral pretibial and ankle edema. Medical history did not reveal any previous disease. Ascites, hepatomegaly, splenomegaly, and facial telengiectasies were present on physical examination. Chest x-ray and CT were normal. Papules and plaques on the knees developed six days after admission. Skin biopsy revealed granulomatous dermatitis. Serum ACE was 250IU/L. Liver biopsy showed non-caseiting granulomas, severe hepatitis and fibrosis. Sarcoidosis was confirmed based on high serum ACE, histopathologic findings of the skin and liver biopsy samples that revealed non-caseiting granulomas.
We report a case of sarcoidosis complicated by cirrhosis as the initial manifestation of the disease without lung involvement. An extensive literature review of sarcoidosis, focusing on case reports, which presented with cirrhosis and portal hypertension without lung involvement, was made.