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Orbit 2021 Jan 4:1-2. Epub 2021 Jan 4.

John F. Hardesty, MD, Department of Ophthalmology and Visual Sciences, Washington University in St. Louis School of Medicine , St. Louis, Missouri, USA.

Medical therapy is the mainstay of initial treatment for periocular xanthogranulomas. Here we depict surgical management of biopsy-proven adult-onset asthma and periocular xanthogranuloma (AAPOX). This 27-year-old female presented with seven years of progressive bilateral periorbital swelling, weight gain, and severe asthma refractory to immunosuppressive therapy including methotrexate, rituximab, and cyclophosphamide. Read More

Medicine (Baltimore) 2018 Jun;97(26):e11143

Clinica Medica, Ospedali Riuniti Clinica Medica Section of Pathological Anatomy, Università Politecnica delle Marche, Ospedali Riuniti, Ancona, Italy.

Rationale: IgG4-related disease (IgG4-RD) is an emerging immune-mediated disease characterized by multi-organ involvement and variable clinical behavior.

Patient Concerns: We describe the case of a 50-year-old woman affected by a rare variant of IgG4-RD, characterized by eyelid xanthelasmas, adult-onset asthma and salivary and lacrimal glands enlargement. Multiple lymphadenopathies and a pulmonary mass were present at initial evaluation. Read More

Skinmed 2016;14(6):449-451. Epub 2016 Dec 1.

Division of Dermatology, Departments of Medicine and Pediatrics, Cooper Medical School of Rowan University, Marlton, NJ;

Am J Ophthalmol Case Rep 2016 Apr;1:34-37

Department of Ophthalmology, The University of Wisconsin, Madison, WI; McPherson Eye Research Institute, Madison, WI.

Purpose: The aim of this study was to report a case of Adult-Onset Asthma with Periocular Xanthogranulomas (AAPOX) associated with systemic IgG4-related disease (IgG4-RD).

Observations: A 57-year-old man presented with bilateral periorbital swelling for 1 year. Histopathology of a left orbital biopsy showed fibro vascular connective tissue inundated with foamy, lipid-laden histiocytes and touton giant cells with lymphocytic inflammation. Read More

Eur J Ophthalmol 2015 Jul-Aug;25(4):288-92. Epub 2015 Jan 15.

1 Ocular Oncology Service, Department of Ophthalmology, Hacettepe University School of Medicine, Ankara - Turkey.

Purpose: Adult-onset asthma with periocular xanthogranuloma and adult-onset xanthogranuloma are 2 rare subtypes of non-Langerhans cell histiocytic disorder and much remains unknown regarding optimal treatment. The authors describe their experience in the management of these 2 disease subtypes.

Methods: This is a retrospective case series with histopathologically proven orbital xanthogranuloma over a period of 12 years. Read More