Sweet syndrome on a patient with autoimmune hepatitis on azathioprine and CMV infection

Abstract

Sweet syndrome (SS) is a rare inflammatory process presenting with painful erythematous skin eruptions, accompanied by fever and neutrophilia. It is associated with upper respiratory infection in fertile women (classic form), malignancy, infections, drugs and autoimmune diseases. Its pathogenesis remains to be determined. Nevertheless, cytokines may have a prominent role, due to a rapid response after corticosteroid administration. We describe a 32-year-old female with autoimmune hepatitis on azathioprine and prednisone, presenting with fever and inflammatory skin eruptions. Histologic examination of the skin lesions showed neutrophilic infiltrations of the dermis, confirming the diagnosis of SS. Concurrently, she tested borderline positive for recent CMV infection.

Case Report of Sweet’s Syndrome Associated With Autoimmune Hepatitis

“…2,3 Despite this known association of AIH and PG, there has been only two previously reported cases of AIH and SS. 4,5 The first report from 1999 was of an 18-year-old woman with recurrent SS who also had elevated transaminases, leading to a liver biopsy felt to be compatible with AIH. This patient had an anti–smooth muscle antibody of 1:320 and a positive antinuclear factor with a titer of 1:1280.…”

“…4 Recently, a second report of the association of SS with AIH was published. 5 This was a 32-year-old Caucasian female with AIH diagnosed 1 month previously who was treated with prednisone and azathioprine. The authors stated that their case met diagnostic criteria for druginduced SS from azathioprine.…”