Refractory periorbital necrobiotic xanthogranuloma

A woman in her mid-seventies presented with progressive, indurated yellow-orange periorbital and facial swelling, and histopathologic examination demonstrated necrobiotic granulomatous inflammation with foamy histiocytes, multinucleated giant cells, and cholesterol clefts, establishing a diagnosis of necrobiotic xanthogranuloma. Systemic evaluation revealed an IgA-predominant immunoglobulin abnormality without evidence of plasma-cell neoplasm, while FDG-PET/CT showed intense uptake confined to the affected facial skin and subcutaneous tissue. Multiple systemic therapies produced only limited or transient benefit, whereas tofacitinib resulted in partial improvement, suggesting that JAK inhibition may represent a therapeutic option for selected refractory cases.