{
  "abstract": "We describe the case of a young adult woman presenting with progressive bilateral proptosis, periorbital soft tissue swelling and eyelid skin lesions. Imaging showed thickened eyelids and enlarged lacrimal glands and extraocular muscles, and a biopsy of the eyelid disclosed a xanthogranulomatous lesion with increased IgG4+ plasma cells. Further investigation did not reveal any associated systemic findings, and the diagnosis of adult-onset xanthogranuloma was made. Periocular lesions and proptosis responded to oral steroids, but symptoms recurred on tapering. Sustained remission was achieved with low-dose corticotherapy. Adult orbital xanthogranulomatous disease comprises a group of rare histiocytic disorders, and pathology is the key to diagnosis. It can be classified into four syndromes with different systemic manifestations and prognoses, adult-onset xanthogranuloma being the least common. Definitive classification is often challenging and requires thorough pathological review and long-term clinical monitoring. Limited evidence is available for the treatment of these conditions, but a conservative approach with immunosuppression is usually considered the best option.",
  "authors": [
    {
      "affiliations": [
        "Department of Neuroradiology, Unidade Local de Saúde de São José EPE, Lisboa, Portugal"
      ],
      "name": "Pedro Sousa Brandão"
    },
    {
      "affiliations": [
        "Department of Ophthalmology, Unidade Local de Saúde de São José EPE, Lisboa, Portugal",
        "Department of Ophthalmology, Instituto Português de Oncologia de Lisboa Francisco Gentil EPE, Lisboa, Portugal"
      ],
      "name": "Ana Duarte"
    },
    {
      "affiliations": [
        "Department of Pathology, Instituto Português de Oncologia de Lisboa Francisco Gentil EPE, Lisboa, Portugal"
      ],
      "name": "Joaninha Costa Rosa"
    },
    {
      "affiliations": [
        "Department of Radiology, Instituto Português de Oncologia de Lisboa Francisco Gentil EPE, Lisboa, Portugal"
      ],
      "name": "Alexandra Borges"
    }
  ],
  "title": "Orbital xanthogranulomatous disease: a rare presentation of adult-onset xanthogranuloma",
  "uid": "0e4c34ce-3632-56a1-bb2b-224b550beb76"
}