Purpose

Adult orbital xanthogranulomatouse disease (AOXD) is a rare non-Langerhans cell histiocytosis characterized by progressively enlarging, yellowish periocular and orbital lesions. It is divided into the following subtypes: adult-onset xanthogranuloma (AOX), adult-onset asthma with periocular xanthogranuloma (AAPOX), necrobiotic xanthogranuloma (NBX) and Erdheim-Chester Disease (ECD). Limited evidence is available for the treatment of the conditions. Therefore the treatment depends on the specific subtype and the extend of systemic involvement. In this case series, we
present two patients treated for AOXD between 2024 and 2025 in our department.

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Methods

2 female; mean age 65 years (61 to 69). Both with a previous history of upper eyelid blepharoplasty. One patient with the medical history of Hashimoto thyroiditis and a previous pneumonia.
All patients presented with thickened eyelids, periorbital soft tissue swelling and yellowish eyelid skin lesions.

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Results

All patients underwent surgical intervention with an upper eyelid blepharoplasty and anterior orbitotomy for biopsy and debulking. In addition triamcinolone was injected intralesional. In one patient systematic corticosteroid therapy was administered and later changed to steroid-sparing immunosuppressive treatment with methotrexate.

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Conclusion

These two cases emphasize the importance of a combined clinical, histopathological and immunohistochemical evaluation. They highlight the diagnostic complexity and challenge to classify the disease in order to make therapeutic decisions.

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Additional Authors

First name	Last name	Base Hospital / Institution
Daniel	Niederstrasser	Hamburg, Germany
Louisa	Michaels	Hamburg, Germany
Ulrich	Schaudig	Hamburg, Germany

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