Abstract
Autoimmune hepatitis (AIH) and Langerhans cell histiocytosis (LCH) are two independently rare disease processes that can have similar presentations. We present a unique, complex case that required a multidisciplinary approach to ultimately diagnose and treat the patient.A 20-year-old male with no significant history presented with worsening jaundice, diffuse, pruritic rash, and abdominal pain over one month. On admission, the patient’s labs showed significantly elevated liver function tests (LFTs), eosinophilia, and anemia. The exam was notable for diffuse lymphadenopathy (LAD), hepatosplenomegaly, and a diffuse, non-blanching, morbilliform rash. Interdisciplinary workup was notable for positive anti-smooth muscle antibody (ASMA) and anti-neutrophilic antibody (ANA). A liver biopsy showed severe inflammation with interface activity, consistent with AIH. A lymph node (LN) biopsy showed findings consistent with LCH, including histiocyte clusters. He was started on high-dose steroids with LAD/LFT improvement; yet, his course was complicated by a gastrointestinal (GI) bleed requiring a hemicolectomy. The patient was transferred to a larger referral center where he continued to improve with steroids and was ultimately discharged.This case was notable for an LN biopsy showing histiocyte clusters with reniform nuclei, nuclear grooves, and eosinophils with immunohistochemical stains positive for S-100, CD1a, fascin, langerin, CD45, and CD68, consistent with LCH. The resected colon showed atypical histiocyte proliferation positive for fascin, CD4, and CD68. Other findings, including elevated LFTs, ASMA, and a liver biopsy showing inflammation with interface activity, eosinophils, plasma cells, and characteristic fibrosis, supported a diagnosis of AIH. In either case, steroids were indicated.
Highlights
Autoimmune hepatitis (AIH) and Langerhans cell histiocytosis (LCH) are two independently rare disease processes that can have similar presentations
LCH is a disease of uncertain etiology characterized by the abnormal proliferation of bone marrow-derived Langerhans cells that can affect any age group but has a predilection for children aged one to three years [12]
LCH is a disease of uncertain etiology that is characterized by the abnormal proliferation of bone marrowderived Langerhans cells, which are myeloid progenitor cells or committed dendritic cell precursors [1]
Summary
AIH and LCH are two independently rare disease processes that can have similar presentations. We hereby present a unique case of a 20-year-old man who was found to have biopsy-confirmed LCH concurrently with biopsy-confirmed AIH Such a complex case required an interdisciplinary approach to diagnose and treat the patient. Image from the liver biopsy (400x magnification) showing severe inflammation and confluent necrosis It highlights the inflammatory infiltrate, which contains numerous plasma cells and eosinophils with interface activity. The pathology results from the resection showed ulceration and chronic active inflammation extending transmurally to the serosal surface and involving the ileocecal valve, cecum, appendix, and ascending colon It showed atypical histiocyte/fibroblastic proliferation positive for fascin and CD68, but negative for CD1a, S-100, HHV-8, pancytokeratin, periodic acid-Schiff (PAS), Grocott methenamine silver (GMS), and acid-fast bacilli (AFB) staining. The patient began to clinically improve, and a collective decision with the family was made to transfer the patient to a larger referral center where he continued to improve with steroids and was discharged with close follow-up
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