Acute Autoimmune Hepatitis with Amyopathic Dermatomyositis: “A Diagnostic and a Therapeutic Dilemma” : A Case Report

Autoimmune hepatitis (AIH) is a rare, immune-mediated inflammatory liver disorder, while its acute form has a spectrum of clinical manifestations including acute-icteric AIH, acute severe AIH (AS-AIH) and AS-AIH with acute liver failure (ALF) (1). A subset of patients with dermatomyositis (DM) presents with characteristic skin findings but without muscle involvement, termed clinically amyopathic dermatomyositis (CADM). This subgroup includes patients with amyopathic DM(AMD), who are devoid of all clinical and laboratory findings of muscle involvement (2). We report a case of a previously healthy 54-year-old woman who presented with acute icteric AIH and features of AMD, rapidly progressing to AS-AIH with ALF and the diagnostic and therapeutic challenges encountered while managing the patient.