[Orbit xanthogranulomatosis. Erdheim-Chester disease]

Area of Science:

• Ophthalmology
• Dermatology
• Pathology

Background:

• Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized by systemic lipid-laden histiocyte infiltration.
• Ocular and periorbital manifestations of ECD can be the initial presentation, necessitating a high index of suspicion.

Observation:

• A patient presented with a three-year history of bilateral upper eyelid edema and xanthelasma-like lesions.
• Ophthalmologic examination revealed orange-yellowish lesions and symmetrical temporal upper eyelid tumors.
• Initial treatment with corticoids reduced tumor size, with recurrence upon discontinuation.

Findings:

• Biopsy confirmed lid xanthogranulomatosis.
• Systemic examinations were otherwise normal, highlighting localized presentation.

Implications:

• Xanthelasma-like lesions and bilateral orbital masses warrant consideration of Erdheim-Chester disease.
• Prompt diagnosis and systemic evaluation are essential for managing ECD and its potential complications.