• Nikita Chhabra, John E Cebak, Alessandra Schmitt, Devyani Lal, Allison C Rosenthal, Cullen M Taylor, Ryan M Thorwarth, Ami A Shah, and Alicia Rodriguez-Pla.
• Department of Neurology, Mayo Clinic School of Graduate Medical Education, Mayo Clinic College of Medicine and Science, Scottsdale, Arizona.
• Medicine (Baltimore). 2022 Aug 12; 101 (32): e30067e30067.

RationaleImmunoglobulin (Ig) G4-related disease (IgG4-RD) reportedly has a strong relationship with adult-onset asthma and periocular xanthogranuloma (AAPOX) and may be linked to sclerosing mucoepidermoid carcinoma (MEC). We present a rare case of IgG4-RD and AAPOX occurring in a patient with resected eosinophilic or oncocytic MEC.Patient ConcernsA 52-year-old woman was referred to our rheumatology clinic in 2020 to be evaluated for suspected IgG4-RD.DiagnosesThe patient had diagnoses of periorbital xanthelasmas, worsening glucocorticoid-dependent chronic rhinosinusitis and adult-onset asthma, and cervical lymphadenopathy persisting 2 years after resection of a low-grade MEC of a minor salivary gland.InterventionsBecause the patient's symptomatic relief was glucocorticoid dependent, IgG4-RD was suspected, and she was referred to our medical center. Her amylase and lipase levels were elevated. Serum IgG4 levels were initially within normal limits, but IgG4-RD was diagnosed because of the presence of lymphadenopathy and evidence of pancreatitis, which was shown on positron emission tomography/computed tomography. Furthermore, the IgG4 levels later increased without explanation. After the patient began combination therapy with a glucocorticoid (prednisone) and methotrexate, her symptoms improved but recurred when the daily oral glucocorticoid dosage decreased below 10 mg. An excisional biopsy of her right submandibular gland in 2021 yielded results consistent with IgG4-RD. In addition, AAPOX was diagnosed, given the presence of periocular edema and plaques, adult-onset asthma, and rhinosinusitis.OutcomeThe patient was carcinoma free at last follow-up and was receiving medication to treat the other conditions.LessonsThe diagnosis of these 3 concomitant, uncommon entities required approximately 7 years of medical investigations. Clinicians should know that IgG4-RD, AAPOX, and MEC may occur together.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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