Suspected Adult-Onset Asthma with Periocular Xanthogranuloma vs. Erdheim-Chester Disease

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  A patient with life-long asthma was referred from an outside optometrist for progressive blurred vision, right eye worse than left, with ~10 years of slowly progressive bilateral eyelid swelling and tenderness. Examination showed bilateral firm, yellow eyelid plaques, mild proptosis, reduced right-eye acuity, and trace right-sided optic disc edema. Orbital MRI demonstrated bilateral fusiform rectus muscle enlargement (sparing the medial recti) and infiltrative superior extraconal soft tissue extending into enlarged lacrimal glands, without cavernous sinus or dural involvement. Serum IgG4 was elevated. Lacrimal gland biopsy showed foamy histiocytes and Touton giant cells characteristic of a xanthogranulomatous process, without necrobiosis. Tissue IgG4+ plasma cells were borderline increased and did not meet criteria for IgG4-related disease. Flow cytometry showed no lymphoma. The clinicopathologic picture was felt most consistent with adult-onset asthma with periocular xanthogranuloma or possible Erdheim-Chester disease. Systemic evaluation including tissue BRAF V600E testing, whole-body PET/CT, and rheumatology consultation was initiated but interrupted by gaps in follow-up. A unifying diagnosis has not yet been established.
  

  Presentation Date: 09/24/2026
  Issue Date: 09/25/2026
  

  • Select activity Presenter: Christina M. Ambrosino, MD

    

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  • Select activity Faculty Discussant: Sander R. Dubovy, MD

    

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  • Select activity Faculty Discussant: Thomas E. Johnson, MD

    

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