We studied the skin of six patients with primary biliary cirrhosis (PBC) to determine if there is cutaneous deposition of complement and immunoglobulins in PBC. We also investigated serum samples from these patients for the presence of circulating antibodies against nuclear and cytoplasmic antigens. Three of five patients demonstrated immunoglobulins (IgM or IgG) or complement (C3) deposition at the dermoepidermal junction (DEJ) in clinically normal, light-protected skin. Two of six patients had 2- to 5-mm papular-pustular skin lesions as a manifestation of PBC. Serological studies in these patients disclosed low antinuclear titers in three patients and anti-single-stranded DNA antibody titers in the two patients with skin lesions. These histological, immunofluorescent, and serological findings offer further evidence of the occurrence of circulating immune complexes in at least some patients with PBC.
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