Autoimmune Hepatitis and Systemic Sclerosis: a Rare Association

Abstract

A woman in her early 40s, with a history of excessive alcohol intake, presented with purpuric, ulcerative lesions on the lower limbs. On examination, hirsutism and generalized stiffening and thickening of the skin were noted. Laboratory investigations revealed hyperbilirubinemia, hypergammaglobulinemia and positive anti-smooth muscle antibodies. Histologic examination of the skin was compatible with scleroderma. Histologic examination of the liver was suggestive of autoimmune and alcoholic hepatitis. LEARNING POINTS Alcohol intake is a main cause of hepatic disease but it is mandatory to exclude other causes. Autoimmune hepatitis and systemic sclerosis may be associated, therefore patients with these diseases should be monitored closely. There exist scores that can be helpful in the diagnosis of both autoimmune hepatitis and systemic sclerosis.