Necrobiotic xanthogranulomas are a rare form of cutaneous non-Langerhans cell histiocytosis.
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Epidemiology
The condition occurs mostly in older adults 2.
Clinical presentation
Patients demonstrate cutaneous papules that develop into large, firm plaques, papules and nodules, which are often yellow 1. The most common site is the periorbital skin 1. Involvement of the periorbital soft tissues can cause ptosis, exophthalmos, diplopia, restricted ocular movement, and pain 3.
Pathology
The exact etiology of the condition is unknown due to its rarity 2.
Associations
Most patients have a monoclonal gammopathy, which has the potential to develop into multiple myeloma or other lymphoproliferative disorders 1.
Radiographic features
Cross-sectional imaging of the face on CT or MRI demonstrates soft tissue thickening or masses in the periorbital region. Internal organs (e.g. the liver) may also be affected.
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