The Great Spiral Masquerader: A Case of Concurrent Secondary Syphilis and Autoimmune Hepatitis

Abstract

We describe a unique case of rash and acute hepatitis confounded by the presence of syphilis that created suspicion for syphilitic hepatitis, a rare and often misdiagnosed condition. Investigation concerning the etiology alternatively lead to the diagnosis of 2 concomitant conditions: active autoimmune hepatitis and secondary syphilis. To our knowledge, this is the first description in the literature of the simultaneous occurrence of secondary syphilis and autoimmune hepatitis. This case serves to increase the recognition of the clinical characteristics and diagnostic challenges of syphilitic hepatitis and to discuss the potential role of pathogens in the induction of autoimmunity.

An Adolescent Female With Concurrent Presentation of Autoimmune Hepatitis and Secondary Syphilis

“…It is a progressive inflammatory hepatopathy involving loss of immune tolerance; while a specific cause remains unknown, pathogenesis likely involves both environmental and genetic factors. AIH may be associated with an infectious trigger ( 2 ). Presentation varies but mostly involves acute or insidious onset of nonspecific symptoms, such as malaise, nausea/vomiting, abdominal pain, and jaundice.…”

“…Thus, AIH was strongly suspected in our patient, but the contribution of syphilis was also considered, even though it may have been an incidental finding. SS rarely (3%) presents with SH, usually with disproportionate biochemical cholestasis relative to normal or mildly elevated transaminases ( 2 ), in distinction to AIH, characterized by transaminase elevation with or without cholestasis. SS is a difficult condition to diagnose given its varied nonspecific manifestations, including nonspecific constitutional symptoms as with our patient, lymphadenopathy, and characteristic rash involving the palms and soles.…”

“…Previous case reports ( 4 ) have implicated SH and its treatment in the activation of an immunologic cascade resulting in AIH. This link has been postulated through the phenomenon of molecular mimicry, whereby Treponema pallidum may activate the host immune system against its own tissues ( 2 ). T. pallidum affects innate and adaptive immune responses.…”

“…Biopsy additionally demonstrated hepatocyte rosetting and emperipolesis (internalized cells), also associated with AIH ( 1 ), and early bridging fibrosis. Further, no histologic features described in SH were noted, such as pericholangiolar inflammation, granulomas, periportal necrosis, or spirochetes ( 2 ), with negative immunostaining for Treponema pallidum .…”