P3113 - Autoimmune Hepatitis Masquerading as Eosinophilia in a Patient with Bullous Pemphigoid

Vincent Wong, MD, Umair Nasir, DO, Asahi Hoque, MD, Kirtan Chauhan, MD, Jianhong Zhou, MD, Jacqueline Forman, MD, David Bernstein, MD
NYU Langone Health, Mineola, NY

Introduction: Autoimmune hepatitis (AIH) is an inflammatory disease that affects females more than males in the second and fifth decades. Patients can be asymptomatic or present with acute liver failure. The diagnosis is made with serological testing and a liver biopsy. There have been several case reports documenting the rare presentation of AIH with peripheral eosinophilia in patients who have Crohn’s disease, ulcerative colitis, Coombs-positive hemolytic anemia, and autoimmune thyroiditis. We will describe the first case of autoimmune hepatitis presenting as eosinophilia in a patient with bullous pemphigoid.

Case Description/Methods: 35-year-old male with a history of atopic dermatitis, bullous pemphigoid, and asthma presented with right upper quadrant abdominal pain, yellowing of the skin, and a pruritic rash. He denied fever, chills, nausea, vomiting, diarrhea, alcohol use, or new medications.



His vital signs were within normal limits. He had tenderness to palpation at the right upper quadrant, scleral icterus, superficial excoriations, and lichenified plaques. Labs were significant for 18% eosinophils, AST 1256 IU/L, ALT 917 IU/L, Alkaline Phosphatase 252 IU/L, Total Bilirubin 6.4 mg/dL, and Direct Bilirubin 2.4 mg/dL. CT showed hepatomegaly and diffuse lymphadenopathy.



Additional lab studies were positive for anti-smooth muscle antibody to 1:320 and quantitative IgG to 3150 mg/dL. Further workup was negative for porphyria, lymphoproliferative process, and DRESS syndrome. An inguinal lymph node biopsy showed reactive lymphadenopathy, and the liver biopsy was consistent with AIH.



The patient’s eosinophilia peaked at 21% and improved on oral steroids. He was subsequently discharged but was lost to follow-up.

Discussion: Peripheral eosinophilia is often seen in patients with inflammatory conditions, autoimmune disorders, parasitic infections, malignancies, or on certain medications. An extensive workup ruled out pertinent causes for our patient such as drug reaction with eosinophilia and systemic symptoms, porphyria, lymphoma, and infections. It was also thought this was a flare-up of bullous pemphigoid. However, his rash was not suggestive, and bullous pemphigoid has not been shown to cause hepatocellular liver injury. The liver biopsy was the determining factor. The presentation of AIH with eosinophilia is rare, and its etiology remains unclear. Treatment is with steroids, which can reduce inflammation, decrease eosinophilia, and improve overall morbidity and mortality.


Disclosures:


Vincent Wong, MD, Umair Nasir, DO, Asahi Hoque, MD, Kirtan Chauhan, MD, Jianhong Zhou, MD, Jacqueline Forman, MD, David Bernstein, MD. P3113 - Autoimmune Hepatitis Masquerading as Eosinophilia in a Patient with Bullous Pemphigoid, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.