Eosinophilic Annular Erythema: A Rare Dermatologic Manifestation of Autoimmune Hepatitis

Abstract

Autoimmune hepatitis (AIH) is defined as an immune regulated chronic inflammation of the liver. Several extrahepatic cutaneous disorders associated with AIH have been described. The most common are a maculopapular rash, psoriasis,vitiligo, impetigo, lichen planus, erythema nodosum, pyoderma gangrenosum, and vasculitis. Eosinophilic annular erythema (EAE) is a figurate erythema which has been associated with several chronic conditions and malignancies. We report the first case of EAE developing in association with AIH. A 22-year-old male with history of acne was referred for evaluation liver enzymes. Six months prior to his initial visit, he developed a pruritic eruption of demarcated plaques on his scalp, face, neck, and extremities for which he saw dermatology. At that time his liver associated tests were normal. Labs were remarkable for eosinophilia (13%) and biopsies of the lesions showed superficial to mild perivascular lymphohistiocytic cell infiltrate with scattered eosinophils in the dermis. He was diagnosed with eosinophilic annular erythema. A trial of hydroxychloroquine was ineffective. Methotrexate caused leukocytosis and worsening eosinophilia, resulting in discontinuation after 2 weeks of therapy. While on methotrexate his liver tests remained normal. Over the next 10 weeks he was treated with mycophenolate mofetil. However, the AST, ALT, and alkaline phosphatase increased to 107 IU/L, 91 IU/L, and 199 IU/L, respectively. Despite moderate improvement in his skin, discontinuation of mycophenolate mofetil was advised and he was referred for evaluation of abnormal liver enzymes. Subsequent evaluation revealed a positive anti-smooth muscle antibody (65 units) elevation of serum IgG (2037 mg/dL) and a liver biopsy showing chronic nonspecific hepatitis with mild interface hepatitis. A diagnosis of autoimmune hepatitis was made based on labs and histology. The patient was started on high dose prednisone with a decrease in liver enzymes. Imuran was added with tapering of the prednisone resulting in both resolution of his rash and normalization of the liver tests (AST 22 IU/L, ALT 23 IU/L, alkaline phosphatase 79 IU/L) Many cutaneous manifestations of AIH have been described. To our knowledge, this is a unique case of AIH associated with EAE. Furthermore, the correlation between adequate treatment of the patient's AIH and resolution of his rash suggests the importance for awareness of EAE as a presenting sign for AIH.