CASE REPORT Primary biliary cirrhosis and Henoch^Schonlein purpura: report of two cases and review of the literature Nikolaos K. Gatselis 1,2 , Angelos Stefos 2 , Chrisa Gioti 2 , Eirini I. Rigopoulou 1,2 and George N. Dalekos 1,2 1 Department of Medicine, Research Laboratory of Internal Medicine, Medical School, University of Thessaly, Larissa, Greece 2 Department of Medicine, Academic Liver Unit, Medical School, University of Thessaly, Larissa, Greece Keywords cirrhosis – immunoglobulin A – primary biliary cirrhosis – Henoch—Schonlein purpura – vasculitis Correspondence George N. Dalekos, MD, PhD, Academic Liver Unit and, Research Laboratory of Internal Medicine, Medical School, University of Thessaly, Papakiriazi 22 str, 41222 Larissa, Greece Tel: 130-2410-565251 Fax: 130-2410-565250 e-mail: dalekos@med.uth.gr Received 23 August 2006 accepted 20 October 2006 DOI:10.1111/j.1478-3231.2006.01402.x Abstract 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. Henoch–Schonlein purpura (HSP) is a systemic immune-mediated small-vessel vasculitis with immu- noglobulin A (IgA)-dominant immune deposits pre- dominantly affecting children and less commonly adults (1–3). Four diagnostic criteria for the diagnosis of HSP have already been established (two necessary for diagnosis), namely palpable purpura, age  20 years at disease onset, ‘bowel angina’ (diffuse abdom- inal pain or bowel ischaemia usually with bloody diarrhoea) and biopsy showing granulocytes around arterioles or venules (1). Adults may present two out of the four criteria of the tetrad (sensitivity and specificity of 87%) (2). The cause of HSP remains unknown, although, there is often an antecedent respiratory tract infection. Many bacterial and viral organisms including streptococci, adenovirus, parvo- virus, Epstein–Barr virus, varicella and mycoplasma have been reported as the preceding factors for HSP (1–3). In this context, the increased incidence of HSP during winter and spring supports the infectious insult in a susceptible individual. In adults, however, the disease is usually presented as a diagnostic and ther- apeutic challenge as it is either a primary-idiopathic disorder or a cutaneous manifestation of an under- lying connective tissue disease, systemic bacterial infection or malignancy (1–3). Here, we report for the first time two cases of 71- and 72-year-old women with HSP in association with previously diagnosed primary biliary cirrhosis (PBC). Case reports Two 71- and 72-year-old female patients with a diagnosis of PBC of 12 and 10 months’ duration were admitted to our department because of palpable purpura involving the legs, buttocks and abdominal wall in the first, and arthralgias and palpable purpura involving lower extremities in the second. In both the patients, the diagnosis of PBC was based on the following criteria at presentation: elevated cholestatic enzymes namely g-glutamyl-transpeptidase (185 and 135 U/l; upper normal limit: 40 U/l) and alkaline phosphatase (204 and 194 U/l; upper normal limit: Liver International (2007) 280 c  2007 The Authors. Journal compilation c  2007 Blackwell Munksgaard Liver International ISSN 1478-3223