[Symptom: ptosis-Diagnosis: xanthogranulomatous disease]

Area of Science:

• Ophthalmology
• Dermatopathology
• Immunology

Background:

• A 65-year-old female presented with recurrent ptosis and eyelid swelling.
• Medical history included Hashimoto's thyroiditis and prior pneumonia.

Purpose of the Study:

• To describe a complex case of adult orbital xanthogranulomatous disease (AOXGD), subtype adult-onset asthma and periocular xanthogranuloma (AAPOX).
• To highlight the diagnostic challenges and treatment strategies for this rare condition.

Main Methods:

• Clinical presentation, histopathological examination (lymphocytic infiltrates, foam cells, Touton giant cells), and immunohistochemistry (IgG4 staining) were used for diagnosis.
• Differential diagnosis considered necrobiotic xanthogranulomatosis and Erdheim-Chester disease.

Main Results:

• Biopsy confirmed AOXGD, subtype AAPOX, with evidence of IgG4 association.
• Initial treatments (blepharoplasty, triamcinolone, corticosteroids) were insufficient.
• Methotrexate treatment led to significant regression of swelling.

Conclusions:

• Accurate diagnosis of rare xanthogranulomatous eyelid lesions necessitates a multidisciplinary approach.
• Combined histopathological, immunohistochemical, and clinical evaluation is crucial.
• Immunosuppressive therapy (methotrexate) can be effective in managing AOXGD.