Autoimmune bullous diseases (ABD), which are divided into pemphigus and pemphigoid, are a series of blistering disorders of the skin and mucous membranes induced by pathogenic autoantibodies. This article reviews and summarizes cases of ABD associated with autoimmune liver diseases (ALD). Sixteen cases of ABD associated with ALD were found, of which 87.5% (14/16) were pemphigoid. Furthermore, 2, 8, and 6 patients had ALD such as autoimmune hepatitis, primary biliary cholangitis, and primary sclerosing cholangitis (PSC), respectively, apart from ABD. Half (8/16) of the patients were male. Tendency of a preceded disease (ABD or ALD) was not observed. In six cases of concomitant ABD and PSC, three had ulcerative colitis complication. Distinguishing ABD associated ALD from a comorbidity of ABD and ALD may be difficult. Thus, the pathophysiology and clinical features of concomitant cases of ABD and ALD need to be further elucidated.

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