Primary biliary cirrhosis and Henoch-Schonlein purpura: report of two cases and review of the literature

We describe, for the first time, two adult patients with primary biliary cirrhosis (PBC) who presented palpable purpuric lesions on the lower extremities and buttocks. Skin biopsy was consistent with Henoch-Schonlein purpura (HSP). One of the patients exhibited renal and gastrointestinal involvement, as well. A favourable outcome was achieved in both the patients (after treatment with prednisolone only in the patient with renal and gastrointestinal manifestations). The possible pathophysiological relationships between PBC and HSP - particularly on the significance of the IgA antimitochondrial autoantibodies - are discussed. To the best of our knowledge, these are the first two cases of HSP, which developed in PBC patients. Therefore, this case study suggests that HSP may be rarely involved in the extensive list of immune-mediated diseases associated with PBC.