Cutaneous immunofluorescence in primary biliary cirrhosis

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Abstract
Biopsy specimens of normal skin of 11 patients with primary biliary cirrhosis were studied by immunofluorescence for immunoglobulin and complement deposition. Of the 11 patients, seven had positive immunofluo- rescent staining, primarily in a granular pattern either at the basement membrane zone or around the blood vessels or in both locations. The principal immunoglobulin noted was IgM, which, in six of the seven patients, was seen at the basement membrane zone or around the vessels (or both). Deposition with C3, fibrin, IgA, and IgG was seen less frequently. In this series of patients, cutaneous lesions included discoid lupus erythematosus, lichen planus, vitiligo, chronic bullous lesions of the legs, and scleroderma. Non-organ-specific immunologic markers in this group included elevated levels of IgG, IgM, and IgA, antinuclear antibodies, anti-nDNA, rheumatoid factor, antimitochondrial antibodies, smooth-muscle antibodies, and circulat- ing immune complexes. We wish to add cutaneous immunofluorescence as an additional immunologic marker for primary biliary cirrhosis. (JAMA 1981;246:1679-1681) PRIMARY biliary cirrhosis is a chronic, progressive liver disease of unknown cause predominantly seen in middle-aged women and character¬ ized by segmental inflammatory de¬ struction of intrahepatic bile ducts. Evidence is accumulating to implicate immunologie mechanisms in its path- ogenesis, including hypergammaglob- ulinemia, the presence of circulating antibodies, immune complexes capa¬ ble of complement fixation, and asso¬ ciation with other autoimmune dis¬ eases. Of the autoantibodies that have been demonstrated in the sera of patients with primary biliary cirrho¬ sis, the most clinically significant is the antimitochondrial antibody, which has become the basis of a widely used diagnostic test for pri¬ mary biliary cirrhosis. Antimitochon¬ drial antibody is present in approxi¬ mately 95% of patients with primary biliary cirrhosis.1 In one series from our institution,2 elevated immune complexes were found in 60% of patients with primary biliary cirrho¬ sis. Immune complex disease fre¬ quently produces immunoglobulin and complement deposition of unin- volved skin of patients, as in systemic lupus erythematosus and rheumatoid arthritis.14 We studied 11 patients who had primary biliary cirrhosis to determine if cutaneous immunofluo¬ rescence would provide an effective immunologie and clinical marker for the disease.

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